History

Journal History

Sunday, October 7, 2007 7:26 AM CDT

It's hard to believe that 4 years ago today, our baby was taken from us and we were left without him, our everything...the sun to which we revolved around. Time does not heal all wounds. The memories remain just as painful today. Who knew the heart could hurt so much. We are blessed to have found some form of normalcy again, nothing like we once knew it, but some form of it nonetheless. Please continue to lift us up in prayer, and all of the Krabbe and Duke kids/families, and the doctors and nurses who give their all trying to help them.

Sunday, May 6, 2007 6:59 PM CDT

Well, we made it to the 2007 Rainbow of Heroes Walk at Duke. It was a real last minute decision, but we made the right choice, and we are glad that we both made it this year. It was beyond fantastic to see so many familiar faces, but I do have to publicly announce our disappointment that we did not see Bobbie or Jeanne, 2 of Noah's nurses at the walk this year!!! So Emily or Cindy, if you read this...make sure you pass along a Prichard scolding to them both because we were really wanting to see them as well! It was so great to see Dr. K again...she has not changed a bit...and Dr. Martin, Dr. Szabolcs and Gil and Sue and June and Marion and Andre and Mary...so many, many of the staff that took special care of Noah. Despite our struggle there, and bumping heads along the way with a few of them, they are all so VERY, VERY special to us and we adore them all. It is hard not to endure what we did, and be there for that length of time and not build a special friendship with so many great folks whom we will always remember! The weather was a bit icky, raining on us, but it still turned out to be a super day. We visited with Kelly, from Hunter's Hope, and Ashleigh Gwin and her family/friends...we had such a great time. The drive up was long...and back as well...but well worth it. We are so glad we went. It is not exactly the easiest place for us to be...there is a lot of anxiety that comes with visiting Duke and being around familiar sites in Durham...old memories flood and overwhelmed us, but we managed, and I think it will be easier each time we go back. Noah has left such a mark...what an amazing baby. An angel...heaven sent!

Thursday, March 1, 2007 7:13 AM CST

Hey Everyone,

Just wanted to hurry and post this to Noah's page. Please, please, please...read below and follow the links to show your support the Illinois bill to do newborn screening for LDS, which will include Krabbe disease!!!! Hurry, hurry, and tell everyone that you know!!!!!!!!!

Thank you,

Debbie, Greg and Angel Noah

**********************************************************

Good news! Right now the state of Illinois has a Bill-SB1566-pending in its State Senate Public Health Committee that includes legislation to add Krabbe disease to the Illinois Newborn Screening Panel. We need everyone's help to get this Bill passed through this Committee by March 15th. Please click on the following link http://www.huntershope.org/illinois.asp as soon as possible and follow the instructions to contact the Illinois State Senate President and the Illinois Public Health Committee via email to offer your support to help get this landmark legislation passed as quickly as possible. Please, do this as soon as you can, the time is now! And with all of us working together, along with the rest of the coalition, we will all help the children born in the future with Krabbe disease and some of the related Lysosomal Storage Disorders.

Please act quickly, click here now http://www.huntershope.org/illinois.asp

Below is a summary of the Bill.

Illinois SB1566

This bill introduces Lysosomal Storage Disorder (LSD) Newborn Screening to the State of Illinois by screening newborns for Krabbe, Pompe, Gaucher, Fabry and Niemann-Pick diseases. Treatment for these diseases is only effective if it is started before symptoms appear, so newborn screening is appropriate and necessary. The rate of incidence for all LSD¹s in the human population is 1 in 5,000. Within Illinois , this means that approximately two kindergarten classes of children are affected each year by a potentially fatal LSD. LSD¹s do not discriminate with regard to sex or race.

Additionally, the bill will protect the monies in the Metabolic Screening and Treatment Fund. The Metabolic Screening and Treatment fund is used for newborn screening to detect rare or fatal diseases in babies. As you recall, Governor Rod Blagojevich took $3.4 million from this fund in 2004, which delayed for three years the purchase of a computerized record-keeping system in the newborn screening laboratory. Additionally, it slowed down the implementation of Cystic Fibrosis newborn screening within the State of Illinois .

This Bill SB1566 also calls for the protection of the Genetic and Metabolic Diseases Advisory Committee. Currently, this advisory committee provides intellectual guidance to the Illinois Department of Public Health by recommending which future disorders will be included in its newborn screening test profile. Disorders are included in screening if a condition is relatively common, testing is simple and low cost, and effective treatment or intervention is available. Currently, however, there is no protection for this committee and it can be dissolved at any time.

As a member of the Senate Public Health Committee, I ask that you support this bill in its entirety and inform other senators of its importance to the protection of the citizens of Illinois .

Ms. Kelly Brown

Director of Family Support and Services

Hunter's Hope Foundation www.huntershope.org

LOCAL OFFICE:

PO Box 3123

Durham, NC 27717

919-620-7850

CORPORATE OFFICE:

PO Box 643

Orchard Park, NY 14127

716-667-1200 or 877-984-HOPE

Friday, October 13, 2006 8:17 PM CDT

Another anniversary past, and I survived. THis anniversary is always one of the worse ones by far, of course, as it is the anniversary of his death; but each time I near the end of his baby scrapbook and view the photos of him in the PICU all tubed and wired and bloated from the more than 7 liters of excess fluid he had in him, I begin to think that God was most merciful to take him when he did, and that Noah is no longer suffering as he did his entire 12 months and 3 weeks of life.

The days seem to be passing quickly, which is nice, as we are eagerly awaiting Greg's homecoming this winter. I think with the weather changing, and the stores putting out their Christmas decorations already, that seems to add to the effect of the time passing quickly. It helps the "light at the end of this deployement tunnel" to finally come into the view. We still have several weeks left, and we are still tightly crossing our fingers for his safety, but it is not as daunting now as it was many months ago. A year is a very long time to be separated from a family member...and not just separated, but knowing that you may not see them again sort of adds some anxiety and grief to the mix. We have been very blessed thus far. Thank you again to all of you faithful followers who have continued to offer your support on the guestbook with messages. We are so grateful and so amazed that even after all these years, you continue to offer us such inspiring support. Thank you. It truly means a lot to us. We don't write as much here...not as much time as we used to at Duke...while we sat idly in a hospital room all day...but we do try to at least once every 1-2 months if we can. We are doing well, considering, and so much has happened in the last few years...it is amazing! We keep talking about writing a book about it all...mostly our time with Noah and Krabbe and transplant, and Greg being the writer that he is, I think he could do a pretty good job at it. I don't know that it would be a book that someone would want to publish, or that others would want to read, but I can't help but wonder that maybe his story could continue to touch other's lives in some way...whether it be a family whose child is diagnosed with Krabbe or going through transplant and facing some challenges, or educating folks about Krabbe disease and what it is like to go through the transplant process, or even simply help inspire someone to be a bit more thankful for what they have or for simply being alive. Who knows...it is just a thought.

Tuesday, August 22, 2006 8:14 PM CDT

Hello everyone, it has been a while since we have updated...another September draws near...another birthday Noah won't have...but another year he does not suffer. I like that country song by the band Diamond Rio, "God Only Cries For the Living"...some of the words are very true...if you have ever listened to it...I always think of Noah...how much we wanted him here with us...how much pain we carry...how much we miss him...but the words of that song bring my thoughts to him...no more suffering, no more pain, no more meds, tube feedings, blood draws, surgeries, ventilators, tests, studies...no more...just peace...just rest. Did you know that is what "Noah" means...it means "peaceful rest". Greg and I are pretty much passed the hard days of grieving...they pop up every now and then, but it is usually sporadic, and brought on by a sad song, or story, etc. We can say "I miss Noah" without falling to the ground hysterical...or getting sick to our stomachs...or the heart aching so bad you can feel the palpitations...have you ever truly felt your heart ache? I have...it is undescribable.

Today, I was at work, doing my usual, predictable job in the recovery room...low stress...Monday through Friday...the 'sham job' of nursing (compared to the Pediatric ICU where I had worked for 4 years)...anyhow, I was recovering a patient who had a minor procedure and on the back of my name badge, I have a clear plastic picture holder, with a pic of Kyan in it. He asked me about him, and if my husband was in the military....etc....then we started talking about him being deployed and that this was his first time for Operation Iraqi Freedom because when his other unit deployed from Hawaii, we were with our son at Duke. Our conversation continued as he asked me to describe what illness Noah was transplanted for...I stated he had a 'rare genetic disease that caused the myelin to break down and his nerves, being exposed and no longer insulated, would become permanently damaged/destroyed.' He interrupted me with a question, asking "Was it Krabbe?"

Imagine my surprise, as I could not swallow, speak and nearly fell out of the chair...it doesn't happen very often to find a person who knows about this disease, and we, much like many other parents, can feel very isolated outside of our circle because it is not known or understood by so much of the public. I could not concentrate and my voice began to quiver as I responded with the question, "How do you know about that?"...as if I needed to ask...most always it is because some child has died of it or is dying of it. He shared his story, his son having died of Krabbe as well. I could not believe it. I found it extremely hard to focus on my job and everyday routine became the impossible task, as I fumbled through the paperwork and process of charting. It is so weird...sort of twisted...to get 'excited or wound up' because I have met someone who has a story as tragically as our own...not excited in the happy sense...it is a terrible thing, but I found someone that has an inkling of an idea of what we went through...a common ground...for a brief moment...I no longer felt so isolated. I don't speak a whole lot about Noah at work and our ordeal. In Hawaii, when we came back, everyone had pretty much followed our story at Duke and was well aware of our journey before, during and after Duke. But since moving...I have felt so isolated...I have to sit and tell the long story of what we have been through as the root cause of why I am having a bad "Noah" day...at work in Hawaii...my co-workers pretty much assumed...it was just a Noah day and I needed space and time. Anyhow...enough rambling...it was a weird day. On a positive note...he mentioned that they had since adopted and have a beautiful child...another commonality and happier ending to a tragedy. Kyan is our joy...a monster at times...a cute monster...and a joy. We are so very blessed! Every day, we give thanks to God...Noah is our angel, Kyan is our boy...and we are a blessed family.

Wednesday, May 24, 2006 9:05 PM CDT

Hello, I wanted to offer much gratitude to those who were able to donate and wish me well for the May 2006 Rainbow of Heroes Walk for the Duke Pediatric Bone Marrow Transplant Family Support Program....wow...that's a mouthful! It went SOOO well...I was able to raise over $800 with your help! Thanks so much! And overall...there were over 700 people in attendance and over $170,000 raised for the family support program to help the bone marrow families...both a new record!!! It was such a wonderful experience to see so many familiar faces and sights. I was so blessed to be able to visit the PICU to meet Daphne, Jenny and Collen...it was so wonderful to be able to meet this special family and precious angel. Please keep them in your prayers. I believe they are back in South Africa now, and could really use all of the prayers that you can muster as they grieve their great loss.
I got to see miss Ashleigh, Michelle and McKenzie; Kelly, Brian, and Cecelia, and SOOOO many others that I could never name them all here...and many new folks too. It was emotionally wonderful....sad and happy....it was awesome. Next year I hope that we will be able to go as a family.

I want to mention that I will be participating in the Susan G Komen 3-Day/ 60-mile walk in Atlanta in October...and am accepting donations so that I can meet the required minimum amount to participate of $2,200! It seems like a lot to me, but I know with your help I can meet this goal, and maybe even surpass it! All funds will go to help increase awareness, education and research for breast cancer. If you think you can help, please visit the web page

www.the3day.org

here is the Breast Cancer walk information. It is a 3-Day Walk/ 60 miles…and I have to raise $2,200 dollars in order to be able to participate…kind of weird I know, but I think it is to encourage those who are really dedicated…and improve donations overall, of course, because if they require a minimum…then everyone who really wants to do the walk is going to raise at least that much….ya know? Anyhow….so….I have my first $90 already….and need only $2, 110. So tell everyone you know to help out if they can. Donations can be made online or via mail by printing out a donation form and mailing it in.

Go to the web site www.the3day.org

Click on DONATE

Click on ATLANTA (October 20-22, 2006)

Type in: DEBBIE PRICHARD and click on SEARCH

Click on: DEBBIE PRICHARD

Then type in donation amount and click on: CONTINUE and follow the instructions

OR…..click on PRINT DONATION FORM to print out a form.

Remember to include on any checks mailed in, MY NAME and PARTICIPATION NUMBER, which is #131343145

Wish me luck...tell everyone you know...relatives, coworkers, friends, neighbors....ask anyone you can think of to help out! Its for a great cause. I will be walking in honor of my mother...a magnificent woman who is a breast cancer survivor. God BLess!

Saturday, March 25, 2006 6:36 AM CST

To my beautiful son...from Dad

I loved you before I ever laid eyes on you

When you were first born I held you tightly and wondered at the miracle before me

On baptism day I carried you to the alter and had to fight to keep the tears away

I cradled you and brushed your lips, you gifted me with your first smile

Long nights without sleep, you were in my arms, I couldn’t calm you pain

I carried you to the doctors and they could not help, they looked at you puzzled and confused, no advice, no cure

You were in my arms when we were told there was no hope, to hold you hurt me so, your father, helpless to save his only son

I carried you to the plane, as we traveled a world away, hope again in my arms

We celebrated your first birthday, I thought we had won; with outstretched arms, I lifted you to the heavens

Two weeks later, I could only hold your hand as the machines rattled away, beeped and buzzed, the science fading fast, the ventilator display our worst enemy

They wheeled you down the hall way, your mother fell with grief; I caught her, but my strength was sapped

I held your hand and kissed you check as you turned cold and grey, my life passing away with yours

My son, tears can not wash away the pain of that day

And now I hold you only in my heart and memory

I miss holding you so, and can’t understand why… you had to go

Wednesday, February 22, 2006 9:15 PM CST

It has been such a long time since Noah's page was updated...but I saw a rainbow today on the way home from work...and of course, thought of our precious Noah. I miss him everyday...still having my "noah days"...when I am brought to tears from heart ache, painful memories, and not having him in my arms. It seems like it has been so very long...TOO long...almost like a bad dream at times. It is weird. I don't mind the tears though...they fall out of love...I am just glad that the anger I first had with grieving has gone...I am left with A LOT of questions and of course, sadness, but that is much better than being angry. It helps having Kyan here...his laughter has brought us so much joy...this blessed child of only a few years bringing us so much spiritual renewal...it is amazing. It is actually fun to dream and live again. Noah is missed...everyday...not a day goes by without my thinking about him and how much he impacted our lives. His transplant day was this past weekend...3 years ago he received his donor cells and we began our prayers for new "non-Krabbe" cell growth. There are a lot of milestones/anniversaries of his transplant journey passed this one that I care not to celebrate or remember, but I know they are coming...every year...they stick out in my mind, like reliving clips of the nightmares we endured at Duke. It was however, parts of his life, his precious life, days that I would not trade, because at least we were together during it all, as a family...my precious baby, still alive in my arms. It is hard to let go...moving on sucks, but it means you are "moving on" without the person you love. Noah, you are loved so very, very much, and missed every day.

The 6th Annual RAINBOW OF HEROES WALK to raise money for the Duke Pediatric Bone Marrow Transplant Family Support Program is MAY 6th, and I am thinking about going to Duke this year to walk in Noah's honor. I walked for him, while he was still inpatient at Duke, but we have not returned to Duke since leaving in October 2003, following his death. But, if circumstances permit, I plan to attend this year in his honor, and am asking that anyone willing sponsor me with donations, to please email me. Donations can be made in the form of check made out to Duke University (in the 'Memo' block please write 06RH/Team NOAH) or in the form of Credit card on the secured web site at

www.rainbowofheroeswalk.org

and typing in Team NOAH at the bottom under team name. The funds will go to help buy food/meals/parking passes for the Duke PBMT families, pay for rent/hotel costs, and for art supplies/games/videos/DVDs and stuff needed in the PBMT unit activities room. They were very helpful to us and everyone on the unit when we were there for 10 months...we cannot thank them enough for all that they did to help us out during our time of need. When we first arrived by Air Evac with Noah, and he needed some Ativan/Valium to help him, our insurance had not switched over from the Pacific branch to the Atlantic branch (it makes a difference for military insurance as to which zone you are in) and they would not yet cover any prescriptions...they took money from the Family support fund to help us pay for Noah's prescriptions when we first arrived. So if you can help, please do. Its a great cause.

Thank you,
Debbie

Thursday, December 22, 2005 10:32 AM CST

Greg's web page has been updated, he has not had a chance to get his internet up and running yet, but I wrote an update to let you know where he is and what is going on. Thank you for your continued support, thoughts and prayers. It is greatly apprectiated.

Debbie

Wednesday, November 9, 2005 1:21 PM CST

Hello friends. I have talked to the caring bridge people and they have allowed me to set up a site so that all of you can follow me in Iraq. The site is

http://www.caringbridge.org/cb/viewHome.do

I had to emplace some security measures; we don’t want the bad guys to have access. You will have to register and use a pass word. There is not much there now just the shell but I will get it going this weekend. Please protect the password…there has been instances of violence against soldiers families because of information posted on the web. If you would like the password please e-mail me at

prichardgs@yahoo.com

this is the e-mail account I will be using in Iraq.

Peace
Greg

If you can't get to the page go to caringbridge home and type in

gregprichard

at view a caringbridge site.

Sorry if I messed anyone up.

Wednesday, November 9, 2005 1:03 PM CST

Thursday, September 1, 2005 11:40 AM CDT

Hello everyone…back from oblivion. I know it has been so long since we last updated and my last entry has lingered for far too long. Kyan is doing well, his English is taking off and with it his personality really shined through. I have been very busy. Our unit will be deploying to Iraq some time this winter. Debbie is not too thrilled about it as I am sure you can imagine. In today’s Army it is only a matter of time before you go and it is now my time so I will go. I hope for a quick year and safe return. Thank you for your continued visit to Noah’s site. He is on my mind always and I miss him no less today then yesterday. I know he will be riding shotgun with me in Iraq.

Thursday, January 20, 2005 9:30 PM CST

I have not written a while in Noah’s journal. For now I am just going to free write to my old fried that floats in space as 0 and 1’s known as Noah’s Caringbridge site. They say that time heals all wounds but I have to be honest I do not receive much comfort from that old wise tale. Noah’s been gone now over a year but to me the grief is as bad as the day we lost him. Instead of crying at work I seem to be able to get it out of the way on the drive to work, why cry in public when you can do it in the bathroom or in the privacy of you own home while your wife’s at work? It is a burden I carry with me daily. Time has only made me better at hiding, or functioning with my sorrow, but in truth it never really leaves me. I recall so much, but unfortunately it is all so bad. Over and over my mind replays the loss of my son and it is very hard for me to stop. Noah and I had a very special relationship it is hard for some people to understand that. Often I hear people say, “Poor Debbie, how hard it must be for her to lose her baby.” There is no doubt that Debbie is suffering, I see it often in her, and I hold her when she cries, but Fathers suffer too. When they handed me Noah after his birth, I felt like God had reached down from heaven and touched me. Can you possibly imagine how powerful a feeling that is? My entire being was rocked; Noah shook me to the core. This is my son, what a gift, what a blessing, I am looking at all I could ever hope to achieve in life, and I am holding it in my arms, my most beautiful boy. It was the best day of my life, there is none that can compare. But within hours I sensed that something was wrong. I tried very hard to convince myself that my feelings were off beam, I tried to explain away what my heart was telling me, science was reassuring me that he was fine, but my heart told me I was going to lose him, and it has never been wrong. Then science started to balk, and from that day on the grieving process started. The doctors had begun confirming what my heart was already telling me. When they said Noah had to be admitted because he had jaundice I was a mess, it just set my suspicions that something was not right. I cried and cried as I held him. It was so bad the nurse handed me a glass of water and tried to get me to sit down. I know they must have been thinking, “It’s just jaundice calm down.” But to me it was the beginning of the end, I was devastated. Noah was not often out of my sight or arms. The few times he was in day care I stole away from work in the middle of the day so I could hold him and make sure he was all right. At home I held him always, we were so connected. I was able to make him smile, and one time, for a brief moment, he even laughed for me. For Noah those were events that could be counted on one hand, and they were only in my arms. Can’t get him to eat? Give him to me I can do it, this is my son and I am his father, I can will it to happen. There was nothing I could not do with him and if he were sick I could, through sheer force of will, make him well, or so I thought. Honestly, I thought I could get him through even as we watched him die. I would not, and could not, lose my son. How selfish and conceited was I, he suffered because of my own ignorance. Now that he is gone I am so lost. I feel like I am in a slump, but it never ends, like the movie Ground Hog Day, the alarm clock sounds and the same day starts over again. I miss him the same as I missed him over a year ago. The day after he died I held Debbie sobbing and told her I could not go on without him. She was so amazing, so strong, and I was so spent. To this day though, I struggle without him, when he died, part of me went with him. I miss him so much.

Thursday, December 9, 2004 1:20 AM CST

So much has happened since we last updated. We began the IVF process, but after retrieval of 17 eggs, learned that only 2 could be biopsied. According to Dr. Vu, our IVF doctor, he feels that although he had given Debbie the average dose of medications, they did not seem to work as well as they were expected to, thus the eggs did not mature enough to fertilize, and only 2 grew enough to be biopsied...so basically it was a failed IVF. We chose to freeze those two upon the advice given by Dr. Hughes, the Geneticist, because he did not feel that our odds were going to be very good with only two. He recommended another cycle of IVF, with medication doses increased to help increase the probability of more eggs fertilizing this time, and thus increasing the number that could be biopsied for genetics testing. So, after taking a month off to allow Debbie's body to rest, we will begin back up in January with another cycle, hoping to implant somewhere around the end of January/beginning of February...hopefully. It is another devastating blow... another chapter in our book, I suppose. We are praying that this next round will work out better. We continue to pray...hard...and question why all of this must be so difficult for us.

Sunday, November 7, 2004 8:33 PM CST

It has been awhile since we posted on Noah’s site, but we check in and read your entries often. We have started the IVF process. Debbie has been giving herself shots every night to prepare for egg retrieval. I know this all might sound crazy to you, doing an IVF and adoption all at the same time but there is a reason behind it. We decided that we had two options when it came to building a family, give up or move forward. Anyone who knows us knows we are not the quitting kind, so the question was how are we going to build this family? After long discussion we decided that we could not place all our eggs in one basket. So we attacked this problem on three fronts figuring we would have to be successful on one of them. The first plan of attack was IVF through the Army. Well the IVF doctor has been difficult at best to deal with and it is looking like that option is going to fall through. So we move to option two, adoption. This was a gift that really came out of nowhere. We had looked into it but the cost seemed overwhelming, and then one of Noah’s loyal site visitors got us in touch with His Kids and the ball started rolling. We are pretty sure that this option is going to work. It took hard work and lots of penny pinching by the entire family, and now we are just waiting for the judge to sign off on the contract to make it official. Kyan should be coming home soon…a blessing for all of us. The third option was IVF through a private doctor. We are working that end right now. We have a wonderful IVF doctor named Dr. Vu and the PGD portion is being done by one of the best genetics doctors in the country, Dr. Hughes, in Michigan. The PGD requires some gifted lab technicians with special skills. Dr. Vu has offered to pay to fly one of these lab techs from California to Hawaii to perform the cell retrieval for Dr. Hughes. This saved us a considerable amount of money, and we are thankful that God led us to such a compassionate and caring team. If all goes as planned we could even have twins, and within the year go from no children to three! A lot of people often say to us, “Wow that will be a lot of work.” To us we can’t see that it would be any harder than taking care of Noah, and we loved every exhausting minute of that. We are determined to build a family and with God’s help we seem to be off to a good start.

Thursday, October 7, 2004 2:49 PM CDT

As most of you know a year ago today our beautiful boy broke free from his cocoon of sickness, spread his wings and flew away. For his entire life we peered through that ugly covering called Krabbe at the beautiful little boy trapped inside. It was that boy that motivated us to kick, scratch, and chisel away at the thick black coating in an attempt to free him. We beat and tore at that black cocoon night and day until our fingers were raw and we were consumed by total exhaustion. However, the more we tore the thicker and stronger the coating seemed to get, until it was hard to see our son through all the black sinews that surrounded him. In the end we were left holding that broken cocoon, staring at it with teary eyes, our vision so blurred we could not see him spread his beautiful wings and take flight. We miss him very much.

Friday, September 24, 2004 2:41 PM CDT

You can visit Kyan's page at caringbridge.org/hi/kyan all the photos we have are on the page now. Hope you enjoy them as much as we have. He is just perfect!

Thursday, September 23, 2004 3:34 PM CDT

WE HAVE PICTURES!!!!!! I am at work now but will be setting up a web page for Kyan this evening and posting his photo. He is just beautiful! What a blessing!

Monday, September 20, 2004 3:10 PM CDT

We got together a big box of goodies this weekend full of clothes, shoes, toys and a tooth brush and sent it off to the adoption agency for forwarding to the orphanage in Taiwan. Kyan will be the best dressed kid in the orphanage that is for sure. We have been working on his room transforming it from an infant room to a toddler room. We picked him up a race car bed and Debbie spent an entire day sorting through and organizing. He is going to have a NASCAR room. We have been trying to learn some very rudimentary Chinese, so far we can say Hello, Mother, Father, and Son, not a lot, but a start. It is exciting to be preparing for a child again. As soon as we get a picture we will be sharing it with all of you. Please continue to pray for a smooth legal process, and that the birthmother will have comfort in her decision.

Thursday, September 16, 2004 2:09 PM CDT

Today’s your birthday son. It marks the passing of another year since you came into our lives and changed us for ever. As we look back it seems we have not seen you or touched your beautiful face for years…you have been gone for so long. Not a day goes by that you are not in our hearts or on our minds, our conversations always turn to you, the rainbow after the rain reminds us of you. We miss you Noah, we always will, you were the best of both of us, you taught us much and suffered even more. You showed us what love truly is, what it means to be parents, the importance of family and the gift of health, to take each day for what it is and face the next with courage, to never take a moment together for granted, to love like there is no tomorrow. We are better parents and people for knowing you, but it does not ease the pain of being without you. We would have gladly traded all we had to keep you here even our own lives. We know that you are well now in the arms of Jesus, but we would prefer to have you in our arms healed and happy. We will send you our prayers and messages on the wind with balloons today. Happy birthday son, we miss you so much.

Love Dad

Tuesday, September 14, 2004 9:27 PM CDT

Well I guess we should share the news. We have been chosen for a 2 year old boy in Taiwan. All we know is that he is in good health and that the birth mother had him when she was young and is unable to care for him. He is currently in foster care. We do not have a picture yet, and accepted this offer as Gods will; this child is the answers to our prayers…we both need each other. As soon as we have a picture we will share this miracle with all of you. We will also be starting a web site for him so friends and family can follow us on the adoption journey. Please continue to pray for us, that the birthmother will have peace, and for the speedy completion of the legal process.

Peace

Tuesday, August 17, 2004 1:14 AM CDT

There has been no news…except for the orphanage in Taiwan has accepted our application and liked the photo album and “Dear Birth Mother” letter we wrote. I know you are thinking GREAT!!! It is good news but that is all the news we have…we are left to wonder when we will be selected to receive the blessing of a child. Debbie and I check the e-mail day and night…over and over…hoping and praying for good news. Lately Debbie has been recording Adoption Stories on the Discovery Channel, we will sit on the couch together and watch them...many of the couples have endured some of the hardship we have and so we can relate to what they are saying…It can be depressing though and has a tendency to cause some anxiety. It’s like watching a “Baby Story” when you know you are going to deliver in a week or so…exciting, but a bit scary. Our problem is that we don’t know when we are going to “deliver”; we could be waiting for a long time for a baby, though we pray it will happen soon. So it is tense here, lots going on…many questions…and no crystal ball to peer into. We are waiting…hoping we will finally get a break.

Tuesday, August 3, 2004 0:56 AM CDT

There has been a lot of anxiety in the house the last few weeks. Debbie and I seem to be worrying about different things at different times and then again, the same things at the same time. If that confuses you don’t worry, I think we are a little confused lately too. There is a lot going on in our lives right now, and we are trying to cram it all into a relatively small space in time… as far as life times go anyway. I drop my retirement in a few weeks, we have an adoption packet in Taiwan as I write this, we saw the Army IVF Doctor last week, and we see another civilian IVF Doctor in a couple weeks. All this seems overwhelming at times. I will be retired by September 05, and we have to get this all through before that, and this causes some anxiety. We have waited and tried to be patient, but years can sometimes pass like seconds if you are not careful, and we want a family…so we are pursing our dream on every front, tracking it down, wearing away the seemingly miles of obstacles. To expend this amount of monetary resources this close to retirement probably seems careless to some but, I think it’s time to dream boldly…there will plenty of time to worry about money latter. If we can grow our family, the rest will fall into place. Trying to come up with money does cause anxiety though, and we have spent thousands of dollars in the last couple of months…some how we are pulling it off. The refrigerator is not empty, we can still pay the bills, the only thing empty in this house is our arms, and we mean to rectify that hopefully soon. Plan A, B, or C has to work we can’t fail on all fronts, can we? So we will fight on tooth and nail, put it all on pink or blue…through the dice…and if we lose we will through them again and again…until we win our dreams or crash and burn. For a long time we stood still in our grief and waited for a blessing to come our way, and though we are still in pain, we realized we would have to do something soon or our hopes…would pass us by. We hope to be able to give all of you good news soon, and love a new member of the family like there is no tomorrow.

Tuesday, July 27, 2004 2:41 PM CDT

I know everyone is probably wondering what has been going on since it has been so long since we posted. Are home study went well and it is on its way to Taiwan and should be there by mid week. We will then be waiting for the news that a birth mother has been selected. We are still trying to raise the money, and if worse comes to worse we will have to take out a loan which shouldn’t be a problem. We go on the 20th of August to a private IVF doctor, the Army is moving so slow and with my back injury and pending retirement we are not going to risk missing our chance to grow our family through a PGD (Pre Genetic Diagnosis). SO we have adoption , IVF through the Army and IVF through private health care all going at once….one of those avenues has to bear fruit, and the blessing of a child again in our home. Thank you all, for your continued support and prayers.

Friday, July 16, 2004 2:43 AM CDT

Thanks for all of your continued thoughts, prayers and messages. Just wanted to share with you that there will be a broadcast on FOX SPORTS NET (FSN) Channel on Saturday, July 17, at 10:30pm Eastern Standard Time of Hunter and Jim Kelly....the Hunter's Hope Symposium...about Krabbe...transplants at Duke...etc. It might be of interest to some of you. BUT...please check your local listings, as the broadcast time has changed a few times already apparently.

Also, please remember Ashleigh and her family in your prayers. They could really use some right now.

www.caringbridge.com/nj/ashleigh.ryann

Tuesday, June 29, 2004 2:08 PM CDT

We are patiently waiting for our home study to be finalized. (You all know that’s not true) We have put in for about 25 grants, made arrangements to take out loans if we have to, and contacted the entire family for help. Basically, we have decided that we will find a way to make this happen. If we get the call that a child is waiting for us, we will have to go, there is no way we will be able to wait because of money. So it is now a matter to see if all our efforts will bear fruit. If we get rejection letters for grants we will re-submit, after awhile they will get tired of the Prichard family. We may get discouraged but we are not quitters. Thank you for all your continued words of support and encouragement

Wednesday, June 23, 2004 12:00 AM CDT

We have not posted in some time because we have been so busy. Debbie is working for the government hospital now and is trying to get settled in her new environment. We had our home study for adoption last Saturday, it went very well. We have submitted our grant packages to about 15 organizations to try to off set some of the cost but unfortunately have yet to receive anything but denial notices (I have some problem with this knowing that a family with two of their own children already received a $7000.00 grant to adopt a third and that same organization has turned us down twice now. We did send our whole story, and they know we have no children, so it is hard for me to understand.) IVF is moving forward we are now on the waiting list…One year long waiting list, but we are on it. Unfortunately we are use to the disappointment and do not expect anything to go as it should, as things keep getting pushed back with IVF (the date now for when we are eligible is the month before my retirement), and the applications for assistance with adoption keep getting denied we just shrug our shoulders and look at each other; we expect it, so you really can’t be disappointed. You can’t force any of it and I am beginning to think we are just not meant to have children, the signs continue to lead to that conclusion. So I tell Debbie we will do what we can and just be thankful for each other, after all that is about all we can do right now.

Tuesday, June 8, 2004 9:58 PM CDT

Wednesday, June 2, 2004 10:33 PM CDT

Wednesday, June 2, 2004

It seems so hard to believe that a year ago at this time, Noah was in the PICU at Duke on the oscillator, very critically ill with a grim prognosis. Time has flown by so quickly, it will be October before we know it…a year since Noah’s death. I’m not sure how that anniversary will be for us. We continue to pray and hope that one day soon we can write a journal about being blessed with a healthy child…and then we would have this happy anniversary to remember and look back on. We are trying…every avenue. I remember some of the comments received after sharing our decision about Isaac from several different guestbook authors that we should ‘simply’ adopt. Is it really ‘that simple’…we are finding out ‘no’, not unless you have money. After all, it is money that makes this material world go round, and although we have enough for us to pay bills and live on…we do not have enough in our monthly paychecks to save the needed $20,000 total to make an adoption happen anytime soon. This includes the home study, immigration costs, airfare and lodging, agency fees, foreign fees, passport fees, child visa fee, and post placement fees. Thoughts, prayers and well-wishes only go so far, and they won’t pick up this tab. I am so amazed at how most everyone involved in the adoption process seems to just want to make a buck off of adoption. I mean after all, do you think that a social worker meeting with us three times, walking through our home and writing up a report saying that we are “Good to go as potential parents” should cost $1400? And I can’t understand how ‘someone’ can morally justify charging a family interested in saving a child’s life by forcing them to “buy” him/her out of an orphanage…knowing that what this child really could use most is the love and care from a family. It seems so criminal. I know my complaining about it all won’t change the ways of the world, but it seems that if we weren’t hindered with not having enough money to “buy” us a child…we would not have to worry about ‘celebrating’ Noah’s death anniversary with empty arms or another Christmas without a child. If it is going to happen, it will, but I think at times we are fighting a losing battle…hasn’t fate already tried to tell us twice that we were not supposed to be parents? Why don’t we listen…aren’t we just in for more disappointment and heartache? I sure hope not…but I won’t be surprised if it happens. So, from here…I guess we continue to save all that we can, try and spread out the adoption time line to bide us more time to save funds, and continue to send out letters for grants. So far, we have been turned away because there are so many others like us, who cannot achieve an adoption without the financial assistance of others, thus one foundation has been overloaded with financial assistance applications and cannot accept ours. There are a few others that we may qualify for, and we will have to wait and see if they can help us out to off set some of the costs. Meanwhile, we wait…impatient, heartbroken and frustrated…and so does a child…our potential son or daughter…alone, without a family, in an orphanage. Welcome to our pity party.

Thursday, May 27, 2004 5:05 PM CDT

What is going on with the Prichard family? Well we have a new development. We are working on trying to adopt a child in Taiwan, while we wait fro IVF. We have sorted through the first mountain of paperwork and will start our home study soon. We are excited, and hopeful that this will all work out without any problems. It seems now that God is sending us in this direction, so we will move forward were He leads. Though the adoption agency has waived all most the entire fee, because of what we have been through, it is still expensive…mostly fees required in Taiwan $10,000, to be exact. We will be applying for grants and hope for the best. Please keep us in your prayers.

Tuesday, May 18, 2004 1:39 AM CDT

I know that many of you are wondering where we are. Well we moved, and for two weeks had no internet. IVF moves forward, a few more test and we can start…more test. We will remain patient for the rewards are great. Some times I feel like Abraham, but we all no how that story ends so it gives me hope, and acts as a reminder. Thank you all for the wonderful words of encouragement and support. You have become our family, and we appreciate your compassion. It will be nice to share with you our pregnancy and birth of a healthy child or children when it comes. Your prayers will have helped us realize that day…I have no doubts…He is listening.

Peace

Sunday, May 9, 2004 0:49 AM CDT

We have not written in awhile. It has been a very busy last couple of weeks. The foundation of our house has fallen and so the Army is moving us on Monday. Thought it is bad news it is also good news. The place we are moving to is much nicer, it will be a welcome change for us. While all this was going on Debbie was selected for a government job as an ICU/PICU nurse, which she accepted. At the same time the Army doctors told me that my back was damaged and that I would have to get out of the Army. You all know that was my plan all along, but I didn’t think that the years of exertion had done that much damage. I was, to say the least, a bit taken back when they showed me the MRI. It is difficult for me to realize that after years of physical activity that now I must take it easy, or pay the price. I have to go see the neurosurgeon in a couple of weeks, and pray he will not accept my case. We seem stifled, IVF is moving so slowly. We looked at adoption but the agency we talked to wanted $16,500.00 for adoption. This child is stuck in an orphanage, and they want us to pay more than we have. It makes me wonder about their motives. Are they concerned about finding this child a home, or the money? It is a hard pill for Debbie and me to swallow. We will not give up. There are plenty of children out there that are in need of parents, I just don’t understand why the agencies make it so expensive if they are truly worried about the welfare of the child. I question their motives, children are not to be bought and sold to the highest bidder. We will keep looking, and trying, investigating all the possibilities. Some day, God will bless us with a child, He will bless us because we will not give up, we will suffer the trials, and some day be blessed.

Saturday, April 24, 2004 3:47 PM CDT

Often we walk down the path of life focused on where to place our next step in the hopes of avoiding a fall. Sometimes it is a good idea to stop for a moment and look over our shoulder at where we have been. It is amazing what can be seen during a brief pause. Though our path may have some dark patches, areas obscured and hazed by the effects of time, some of the littlest things can jump out at us and remind us of where we have been and how it has impacted where we are going. I stopped the other day and took a seat near an old knotted shade tree and in a moment of silence gazed back down the trail I had just traveled. I decided to look past the big things that lay in my wake and focus on the not so obvious. What struck me most of all were movies. Yes movies. I’ve seen thousands of them, but remember the details of very few. But what significance did movies play in my journey? None really, but it made me realize that the big events of the past, those clearly visible life changing events on the trail, effect the small events of the future and change the look of everything we see. I realized that of all those movies I had seen, none had any emotional impact on me. I could watch a sad movie and never shed a tear. Turn to Debbie and say, “That was a sad movie.” End of discussion, no affect what so ever. Why, because I had never experienced any significant event during my journey that allowed me to relate to what I was seeing. Yes it appeared sad, but I had no idea what it felt like. As I looked back at movies that I had seen closer to my resting point on the trail, I realized that I had shed quite a few tears these last few months while watching a movie. The significant events of the past helped me to relate to the plots and characters portrayed in the movies. Those images were hitting home, when in the past they would simply roll by acknowledged but unfelt. The journey has been hard and sad this last year and a half but it has given me much. It has helped me to feel emotions I had never felt before and to relate those feelings to others. I showed compassion in the past to others with problems or loss in their lives but now I can also feel compassion, and I know loss intimately. And so my path has changed, what was behind me has given everything in front of me a new look, yes, even something as simple as a movie.

Sunday, April 11, 2004 1:06 AM CDT

On this holiday weekend, I began thinking about those soldiers and military members that will not be celebrating this Easter in peace and safety, at home with their families. So, as a military spouse, having endured deployment only a couple of years ago, I wanted to write down a poem that was published in our Family Readiness Group Newsletter a while back, in honor of all of the spouses and families stateside who will be celebrating this Easter without their military member. God bless each of these military members and their families…the sacrifice is great, much greater than will ever be recognized.

Loving A Soldier
(author unknown)

Loving a soldier is not always nice.
In loving him, you pay a high price.
It’s mostly loving with nothing to hold,
It’s being young, yet feeling old.
It’s having him whisper his love to you,
Then comes a kiss, a promise of love;
Knowing you’re watched, approved from above.
Reluctantly, painfully, letting him go,
While you’re dying inside from wanting him so.
Watching him leave with eyes full of tears,
Standing alone with hopes, dreams and fears.
It is sending a letter with stamp upside down,
To a far away love, in a far away town.
It’s going to church to kneel and pray.
And really mean the things that you say.
And you will wait through rain and shine,
To hear some word that he is fine.
Then the letter arrives and you’re given to joy,
Like a small child with a shiny new toy.
With fingers a trembling and heart beating fast,
You tear open his letter and read it at last.
Yes, he is well, and misses you so;
And it is filled with the love you wanted to know.
Weeks are a month and months are a year,
You’re awaiting the day you’ll have no more fear.
Time passes slowly, yet it’s gone very fast,
You’re barely aware it is here till it’s past.
Yes, loving a soldier brings bitterness and tears,
Loneliness, sadness, and despondent years.
Loving a soldier really isn’t much fun,
But it’s well worth the price when the battle is won.
Remember, he thinks of you everyday,
He is sad and lonely for being away.
So, love him, miss him, and try to be bolder,
And ALWAYS be proud…of loving a soldier.

Dedicated to the comrades in Greg’s unit, the 25th I.D., who have fallen, and their families who mourn the great loss…and to those who will be saying their ‘goodbyes’ this next week as more from the 25th I.D. head out. God be with you! Hooah!! Have a blessed Easter!

Tuesday, April 6, 2004 2:45 AM CDT

Well Debbie’s off to work and I am home alone. As always it is my time to write. I miss her and my sons most when she is away during the night. You could never put a price tag on the time we spent at Duke. Though it was terrible, we were always together, inseparable. And now the world creeps in and steals our time together from us. Time together is a sweet prize, we watch each other's schedule and look forward to the moments we can steal together, away from the world. This time would only be sweeter if it was spent with our children. Our world revolved around Noah, and if we are blessed with another child it will turn again, around that beautiful child. We will have no problem casting the world aside, throwing off the chains, and lifting a child in our arms to greet the sun. We can only hope for that, we pray. And so tonight I think of what has been and what can be, and look to the morrow, to steal time, to beg on hands and knees, to have my time gladly consumed by a child.

Friday, April 2, 2004 0:41 AM CST

Well, to briefly add to Greg’s synopsis of yesterday’s events, we learned today from the resident with whom we had some confusion yesterday that we will be placed on a waiting list after mid-May labs are in and complete…waiting 8-9 months after that just to get started. Our hearts sunk, knowing that this is going to be very hard for us to do. We have no other choice if we want to see this opportunity through, so wait is what we will do and pray during this time that it goes by quickly! One worry is that we will be moving in about 14-16 months from now, and we don’t want to cut it too close to preparing to move. We have a pretty good idea of where we will be moving, and it won’t be near a military hospital that has IVF available…as there are only five nationwide that perform IVF, and no one has done ‘selective’ IVF yet. We want to have it done before we leave here. So it would be even better if they would be able to squeeze us in a few months earlier…so we will pray for this too!! Thank you to everyone for all of your prayers and your show of support these last several months. They have been the worst ever to endure, and we needed this week to pull us up and move us forward. We started the lab process today, by having Greg’s blood work drawn. Mine is a bit more intensive, having to be drawn at specific times during this month, so we will take things a week at a time, praying all the while.

PLEASE VISIT NOAH'S LATEST AND NEWEST MEMORIAL QUILT, MADE IN MEMORY OF HIM ESPECIALLY BY THE QUILTING ANGELS AT QUILTS OF LOVE. (His other quilts made by both Quilts of Love and Smile Quilts can be visited by typing in the links listed above).

http://quiltsoflove.com/memorials/noahp/noah.html

Wednesday, March 31, 2004 10:51 PM CST

I know everyone is on pins and needles wondering how it went today. Unfortunately, we also spent a good portion of the day on “pins and needles”. We went to our appointment this morning with a mixture of emotions spinning around in our heads. As we drove in silence, up the long hill to the hospital Debbie asked me, “What are you feeling?” It was hard for me to describe what I was feeling; scared, anxious, excited, optimistic and pessimistic at the same time. I thought to myself, the journey begins here, but the destination is still an unknown. There is a constant fear in this family of disappointment. We get there, sign in, and are ushered into the resident’s office, ...no waiting?...that is unusual for the Army. I’m thinking; 'well off to a good start'. We meet the doctor and thus began the long list of questions. “What happened with your first pregnancy?” the doctor asked, I thought to myself 'here we go, another attempt to explain the unexplainable'...they never fully understand. We told our story and gave her a pamphlet on Krabbe and then moved on to pregnancy number two. She then had us go to an examination room, did a quick examination and directed us back to her office. She handed us a list of the labs we would have to get done and then asked if we had any questions. This is when things began to take a turn for the worst. “Is Tricare going to cover this, we were told we were not eligible for in vitro?” we asked. “I don’t really know, are they?” the reply, with another question. “Are we accepted into the program?” we asked in dismay, “We will see; there are some questions as to whether or not we can test for Krabbe, and we need your genetic information.” She replied. I was becoming frantic, “You have our genetic information.” “No we don’t.” she returned with a look of puzzlement. I shot back, “The CVS doctor said he personally handed it to Dr. Fraterelli, and that he had already sent it to the lab and they indicated that there was no problem testing for Krabbe.” She stood up and said she would talk to the attending, leaving Debbie and I to stare at each other in wonder. She came back and informed us that the attending did not receive our genetic information and that there was no conformation with the lab. So I am thinking why are we here? She told us to do the tests and come back around mid May, a conservative date for when all test results should be in, and we will see what happens; then she ushered us out of the office. We went to the hall dumbfounded, and called Duke to have them fax our genetic tests to their office. We waited for the results to come in, got a copy and handed it to the secretary. Now what? We had lunch and talked about the visit. The more we talked the more scared we got that there was a problem. Wait till May to be told we can’t do it? No way, they have to be upfront with us, that’s what’s fair. So when we got home I paged the doctor who got us the appointment in the first place and explained our visit to him. He told us that none of that sounded right and said he would page the attending and get to the bottom of it. And so we waited, both of us feeling like we had just been dragged down a gravel street by a team of horses. He called us back and told us we were in, and that there was some confusion on the part of the resident because she had never dealt with this kind of case before, everything is a 'Go', to coin an Army term. So we’re in! Praise be to God! I returned to the office late in the afternoon having spent most of the day tracking down answers and Debbie went off to slumber after working the entire night before. When I arrived at work I was told to call the unit First Sergeant who informed me I was passed over for promotion. Fine, then it is final, I will retire. Children matter most to me, the rest of the world can pass me by. I don’t know what I want to be when I grow up, I have thought about becoming a Pastor, or teaching high school history; I will put it in the hands of God. Debbie and I can survive anything; God will put us where we need to be, and that is always together. Thank you all for the prayers, please don’t stop! It will be over when we hold a beautiful child or children in our arms, God willing.

Friday, March 26, 2004 11:48 PM CST

Oh little boy
Sleep softly in the arms of the night
Moon as your night light
The stars will guide your way
Listen to the silence of the darkness
Pay not attention to our tears
As we wish to hold you this night
My little angel of light
Feel the winds embrace
The softness of the clouds
The freedom of the air
You are here
And we are there
But visit us in our slumber
Let us touch your face
Feel our embrace
Comfort us in our dreams
Oh little boy
The better of us both
The dream of hope

Love Dad

Wednesday, March 24, 2004 2:02 PM CST

Satan lurks in the shadows, and for some reason has decided to attack us every chance he gets. We have been saving because we were told that we would incur some of the cost of in vitro. Well yesterday, someone used our information and hit our checking account pretty hard, the money we had set aside is gone, all one week before we go in. I am having a hard time understanding all this. We will recover the money but it will take awhile, meanwhile our account is a mess. I guess because he can’t touch us he goes for what he can. It is frustrating.

Tuesday, March 23, 2004 1:02 AM CST

I added new pictures. This is how I like to remember my Noah.

Love Dad

Saturday, March 20, 2004 4:34 AM CST

There is excitement in the house. It started like a whisper from a dark corner, and has built to crescendo, rocking the foundation and shuttering the rafters till it seems the roof will come off. We have tried to stifle this excitement afraid to get too caught up in it, fearful of more tragedy, frightened of the heights it might take us before the bottom drops out and we fall. But like a starving man grasping at crumbs from the table we grasp at the crumbs of hope that have fallen before our unbelieving eyes. Tragedy has brought about opportunity. We decided to wait before telling anyone but it has become too much to hold in any more. An in vitro doctor at the Army hospital was told of our case by the doctor that diagnosed Isaac. After some research into our problem and some phone calls from Dr. Kurtzberg he decided to take our case. We will be seeing him on the 31st of this month to talk about a selective in vitro. This will be the first the Army has done and so there are some unknowns and I am sure some hurdles we can not see yet, but it is a start. The doctor has a very high success rate and helped a soldier in my unit to have a son. They called us and asked if we would have a problem having twins. I had to laugh; two children without Krabbe would never be a problem for us but a blessing, we could only pray for twins. So this next week can not go quick enough. We are tired of the waiting and uncertainty. I see the potential for a great miracle, I only hope God does too.

Tuesday, March 16, 2004 6:08 AM CST

An attempt at a poem, written in honor of our angels...we miss them so much. The pain never leaves, we remain its prisoners...set free only by the thoughts and words put down on a page.

How do I count my blessings? Do I add, and then subtract…
Why did this all happen to me? Where did I get off track?

I always felt that I was good, and nice;
Told the truth, helped others, said my prayers, and did what was right.

Do I question God? Do I even dare try?
Will it make him angry with me? Will he even answer my “Why’s?”

My firstborn son, an answered prayer…beautiful, perfect, a mother’s dream.
His eyes and nose, just like mine; he was completely angelic, except when he screamed.

All day he would cry, and we knew it wasn’t right,
Took him to the doctor often, to be told that he was just fine.

Not to worry we were told; just two over-reacting parents.
Take a break, catch a movie, or just drive around the block,
But don’t worry, no, don’t worry, he just has a little colic.

Two days later, to our horror and surprise,
A death sentence read out loud, our beautiful son will meet his demise.
Not infection, not a tumor, but an unfamiliar name,
Never heard of, like a silent killer, our son had Krabbe.

In the weeks and months to follow, a perilous journey did unfold;
Prayers went unanswered, where was God, I’d ask, but was never told.

We kept vigil at his bedside, sleep an unfamiliar friend,
We watched his body weaken, while our spirits depreciate.
Our son’s life and our faith, hanging in the balance…
But, we’re his parents, its okay; we’ll just sit and wait.

Wait for the ‘inevitable’, some were quick to say.
We weren’t fit to make decisions anymore, as our son’s life was circling the drain.
Being told, “His situation’s futile, nothing more to do”;
“We weren’t thinking clearly now”, just so desperate to do more.

So many gave up the fight, weakened half way through.
But four determined souls, kept up the pace, steadfast and resolute.
Led by an infant child, two parents and his physician,
Kept fighting for him to stay with us, refusing to give in.

Then one day at his side, him barely hanging on;
We told him that he had fought hard enough, we were proud, and he could go.
Choking to speak the words, I never wanted to say,
How could a God so loving and so good,
Give first to us this incredible boy,
Let him suffer, and then take him away.

We lost our fight, our son, our hope, our dreams, our faith…it all.
Our entire world was in the life of this boy,
His last breath taken, his body left limp and cold;
I bathed his little body, taking extra special care,
To capture every feature…his face, his nose, his hair.

I will grieve his loss forever, my heart left with a hole;
A special little Noah James, came down and unintentionally stole.
I never could imagine any pain more difficult to bear,
Than to say ‘goodbye’ to my child of just one year.

Just when we thought life was over, and our chances at parenting done;
God blessed us with a pregnancy, this child our second son.
But little did we know, that he too would surely die.
For Krabbe was marked in his genetic code, and in months, would snuff out his life.

What should we do, what decision do we make?
No one can tell us, but we know what’s at stake.
We have been down a road of suffering and uncertainty,
A road that may allow life, but there are no guarantees.
Can we handle holding another dying child,
Can our hearts and our minds withstand another trial?
We love our Isaac Andrew, we wanted him with all our hearts,
But the thought of him suffering like Noah did,
Was tearing us apart.

Can you forgive us Isaac, for making this choice;
My son, so innocent and alive, but without a voice.
Our wounds run so deep and our pain is so strong,
We have made our decision, out of two, that for us, were both wrong.

I pray, Isaac, that you have found your brother,
You are playing, having fun,
And watching out for each other.
I pray each day and night, that you will know and feel our love;
And that you will both understand,
All of our choices were made completely out of love.

Although I will always wonder,
Why bad things happen to good people,
Why children suffer and die, yes, I continue to sit and ponder.
But I do know that the two blessed boys we were graciously ‘given’,
Are two amazing little Angels and have been added to the ‘count’ of blessings in Heaven.

Written with love, in memory of Noah and Isaac, from Mommy.

Tuesday, March 9, 2004 3:38 AM CST

I am so weak, I act like a coward, and there is no bravado anymore in me. I have jumped from helicopters, had guns waived in my face, deployed around the world, and walked the mine fields of Yugoslavia without fear. But now I can muster little courage. I am shell shocked by the last year, and yet driven. I would rather walk a thousand battlefields than deal with the future right now. I look at the pictures of Noah and they tear me apart, I want to be a father again but it seems we cannot get a break. My optimism has become pessimism. I used to be able to see the future; it is all a black cloud now, I can’t see past the hour. A door has opened to us but I am skeptical and scared. You see, to me, every opportunity is a chance for more terror now. I wish I could stand up and say it will be all right, Debbie needs to hear that as bad as I do, but I can’t. As far as I am concerned we have lost two boys in four months, how can I say it will be alright? So what do we do? Wait, ...for what? Adopt? That’s not such a sure thing either, we have looked into it, and sometimes adopted children are taken back, there are no guarantees in the world anymore, I have learned that lesson the hard way. Would that happen to us? Right now I could see that. Will the Army help us? Maybe, but that is also a long shot. So do we try again? Three Krabbe babies in a row, odds are against it, but than again, the odds were against two in a row also. The odds were in our favor with Noah’s transplant but we all know how that turned out, he suffered and died. We will not put another child through that nor could we endure it. We charged in…save our son, we will do whatever it takes, keep going, fight on, but the whole time Noah suffered, never again, never. Yes my feet are stuck in the mud, my soul chained and I can’t find the key to unlock it. Too many prayers have gone unanswered and I can’t understand why. I am tired of praying, of hoping. My hopes have been continually dashed now for over a year. I am beginning to think I know what hell is like, and it scares me. I keep saying to myself we will see what tomorrow brings, but tomorrow is no different that today, after awhile it all runs together. Duke seems a million miles away; Noah has been gone a lifetime, and time is standing still for us now.

Friday, March 5, 2004 0:13 AM CST

I spent some time trying to answer to some of the messages on our web site. We had the site password protected because of some awful entries that are now long since deleted. This week I had to come to the conclusion that answering to others is a waste of my time. Nothing I write will help them to understand, it only gives a reason for more criticism. The site is not theirs it is ours, we bought it with a years worth of tears and agony. The site is our place to write what we feel, not to answer to criticism or out right hatred. The journal is our friend to vent our feelings to, it is the only one we have to talk to, and it always listens. We gave it to the public, we wanted to share this journey, we thought it might help others; we were trying to be unselfish. I did not take lightly closing the door to some, but I knew those who really cared would seek us out. Often I write when Debbie is at work, the house is empty and quiet, there is no plan or reason, I just write. I found that I was reading what I wrote and questioning how others would perceive it, would they understand, would they shoot back? That feeling ruins the integrity of the journal, once you start making adjustments you are no longer writing what you feel. This is not a news article; I do not have to be politically correct, only honest and open. The message received is up to the reader, even if they don’t understand what is going on those that read with compassion reply with compassion, those with hatred or that are ignorant reply likewise. I can’t believe it took me this long to figure that out. So now we are free as long as the password stays protected, that will be your sacred trust as the reader. I can write what I feel; I can talk to my old friend without the ears of strangers listening in. I am through being judged by those who do not know me. We ach too much to deal with negativity, our sense of hope has been utterly crushed, we are lost. I spent the last week wandering around in a daze, it is hard to get motivated, and it is a major chore to go to work, to function, to concentrate. I am tired of feeling this way the events of the last year and last few months have worn me down. Friends tell me “Be strong for your wife” but I am beginning to feel that the yoke is becoming too much to bear. I am so tired, and so is Debbie. We can’t recharge each other anymore and when one is particularly down it pulls the other deeper into the well, it is a cold and dark place down there. We will get through this, we always do, but it is hard. The hardest thing I have ever had to do, make it to the end of the day, I never would have thought that over a year ago. Hopefully a door will open for us soon; we can not bear the thought of losing another child, and we want so desperately to love and hold a child again. Peace

Monday, March 1, 2004 1:25 AM CST

“What would Jesus do?” I have seen this question on t-shirts, key chains, and bumper stickers, the letters WWJD are even embroidered on my Bible cover. How could we ever know the answer to this question? We can ask this question in prayer, but the answer is not always so obvious or clear, it’s not like calling a help hotline, there is not obvious reply. We can study the Bible and see what Jesus did, but it is unknown how he would answer when faced with the issues of the modern world. What counsel would he give us or the transplant team at Duke? I am at a quandary when faced with these questions. How would he act in response to our horrible dilemma? Would he turn his back on us and walk away, point his finger at us in judgment, or post a condemning message on our web site? I can find some peace though in what I know about our savior. He did not hang out with the winners of the world. He didn’t spend his days hobnobbing with the wealthy, the learned or powerful men of influence. He moved in the circles of the needy and down trodden, he was the gospel, the good news to those who needed it most, the ones who listened with open hearts and teary eyes. He witnessed judgment, and replied with grace. When the righteous condemned those around him for their sin, Christ offered them forgiveness, and most importantly unconditional love, like the love a parent gives a child…but more perfect. The New Testament is full of accounts of people being judged by those around them, and Christ continually steps out of the crowd and takes the unpopular stand, he shows them mercy and forgiveness, and in doing so he exposes the weaknesses of their accusers. Some walk away and think about it and others hate him for it, so much they condemned him to death. It’s easy to go with the crowd, remember the mob rules, but Christ could not leave someone alone, popular theory was second to love. If I could meet Christ, if my tears could wash his feet, would he push me aside? Would he study and tear apart my thoughts and feelings openly shared? Would he cast the first stone, to be followed by others? My faith tells me no. It’s unfortunate we all can’t be more like Christ, or know, “What would Jesus do?”

Before you chime in with Christ would never terminate a child, remember he probably wouldn’t transplant one either…he wouldn’t have too. Peace...

Tuesday, February 24, 2004 8:18 PM CST

Debbie and I have asked this question often over the last year, “Where is God in all of this?” it has perplexed us to no end. It took a moment of silence and reflection to figure it out, a pause in the storm. He is still here, just not were we wanted Him to be. He is in the gentle touch of a breeze or the warmth of the sun on our faces. He is in the soft hold of my wife and the love of a pet, the compassion of a stranger and support of a family. He is in the rolling thunder before the cleansing rain comes; He is the full moon that lights our way in the dark, my pillow when I sleep. He can be seen in the blossom of a flower, we can smell him carried on the ocean’s mist. He is in the joy and excitement of reunion and travels on the tears of a goodbye. He is the warm sweater on a cold night, a lost keep sake found. But most importantly He is in our hearts and so never very far away. We just had to take a moment of silence and look.

Monday, February 23, 2004 5:23 AM CST

I received an e-mail informing me that Luther was wrong in his statement "Sin boldly - but trust in the grace of God more boldly still." The argument was backed up with Bible verses which I am aware of, and I am sure Luther was very versed in also. So I thought it necessary to explain the circumstances behind this statement, in case anyone else didn’t understand its meaning. I have spent some time reading Luther’s sermons in German and translating them to English for the Church. I know that may shock some of you who thought I was some uneducated Army grunt, but it’s true. What did Luther mean by that statement? Go out and sin? Do whatever you want, it does not matter? No, he was trying to make a point, and to understand that point you have to know the audience he was addressing. During that time there were some practices in the Catholic Church that bothered Luther. One of them was the buying of forgiveness. If you sinned, then you could pay a priest to pray for you or give you absolution. Some rulers and nobles hired entire monasteries to pray for the forgiveness of their sin. Picture hundreds of monks praying for some noble’s sins to be forgiven for a fee. It happened and was an acceptable practice. So to the common peasant who had no money, there was no hope of forgiveness, he couldn’t afford it. The point Luther was trying to drive home was that you don’t have to pay money to be forgiven; it is through the grace of God. This was a radical statement for his time. There must have been some who could hardly believe it. You mean I am forgiven purely through the grace of God? I can ask for forgiveness myself through prayer? I don’t need someone to do it for me for a fee? Luther said NO, the fee was paid by Christ. He often drives this point home in his sermons because the population needed to hear it. So is he telling them to go out and sin? No, Luther realized people sin; you can stand on the pulpit and tell the congregation not to sin but is that a reasonable request? The point he was trying to make was that we are all condemned by the law but set free by the grace of God through Christ Jesus. Luther was aware that the people he was addressing would sin, but it was important that they know and trust in the grace of God. I used Luther’s statement, because regardless of the decision we make we trust boldly in the grace of God. God alone knows our hearts, and intimately understands us. Peace…..

Friday, February 20, 2004 11:34 PM CST

Luther said "sin boldly - but trust in the grace of God more boldly still."

For those of you who have shown us compassion regardless of your feelings… Thank you. For those of you who felt the need to lecture and judge, you obviously don’t know us, or care about us…I know, you care about the child… but it’s easy to sit at home watching the web page as we watch another child suffer. It is easy to sit on the moral mountain and role stones down on the troubled, but a bit more difficult to climb down from lofty heights to offer them a hand. Those of you who know us… have climbed the mountain to hold our hands and the rest of you…still throwing rocks, don’t forget someone is watching you.

Tuesday, February 17, 2004 1:12 AM CST

Well it is time for me to weigh in. When Debbie told me she posted our story on the web page I must admit I was a bit taken back, but she said she needed to get it out and the journal has always been a good tool for that kind of release. So here is my take on all this. At the end of World War II, young men returned home and tried to “get back” to a normal life and forget about the horrors of war. For many it was a difficult thing to do and inevitably they gravitated back to each other because they held a common experience, they could understand each others feelings, they shared a common bond. Many formed riding groups and bought up the surplus Harley Davidson’s they had ridden and loved while overseas. These first biker groups where really veterans looking for some comradeship and fun. The craze caught on and as America changed in the late fifties and early sixties so did these groups. They began to get a bad rap as derelicts that were living outside of society’s acceptable standards. For the most part the veterans were gone now, much older and advanced in their lives, families, and careers. A small group of bikers began to receive national attention for there ruff lives and involvement in crime. They were known as the 2%’ers, though they received all of the publicity they represented only a small portion of the riding community. So what does this have to do with anything? Noah was brave and strong, and so beautiful. His experience has helped many children that have transplanted after him, and his story has traveled the world because we wanted to share his struggle. Now things are done differently at Duke; children have a better chance because of what Noah taught the team. But Noah was not their first transplant, he was diagnosed relatively early, his prognoses going in was very bright, and he was at the best place, with the best doctors and nurses, money could buy. So what happen? We ask this question often and no one can answer us. What happen? The problem was that Noah was a 1%er, and in many instances the first. He did not represent the typical Krabbe transplant child. He did not respond to treatment the way years of experience and science predicted he should, he went out side the norms, he was a rebel… but not by choice. Veterans of transplant, who recount their war years may have had similar experiences and loss, but their experiences are not like ours, if they were they would be a member of the 1%er club. Noah’s complications and cause of death are still a mystery to this day, his autopsy has produced no answers other than his body was in distress. There are no signs of infection of virus, the killer for many transplant children, and he did not have graph verses host. No Krabbe child has had mixed cells from stem cell transplant, and the consensus among the doctors has been that the few he had would eventually have be killed off. So we ask why again? Unknown… lethal doses of chemotherapy should have done the job, you made a strong baby, he beat chemotherapy, we were told. We didn’t want him to beat chemotherapy we wanted him to live. We were told several times that our child would die because he was not getting better, and no one, even to this day, can tell us why, it is all speculation. In frustration one day I told a doctor, “Don’t tell me you don’t know, it is your job to figure it out.” but they still could give us no answers. We grieved because we would lose him; than he got better (keeping in mind, better is a relative term, it meant he was off ventilation) only to be followed by another set back, again for unknown reasons, again told we would lose him. For nine months we endured this, but worst of all Noah endured it. We put him through transplant to try and save him and in the end he suffered and died in our arms. This is not about Debbie and I, anyone who truly knows us, would know we would give everything we have to save our child. This is about a child, a child we will not allow to suffer, and than die an agonizing death over a year’s time. I have already heard it; Duke has been successful with infant transplants, look at the children, and look at how well they are doing. I ask you, are they members of the 1% club? If they were, those children would be gone and I am sure their outlook would be different. So before anyone tries to change our minds. If you are not a 1%er, and there are none out there, don’t even think about it. Be thankful you are not in our club of three, reduced now to two. And if our decision troubles you, than walk away, we were glad to know you, and we understand, because when the smoke clears Debbie and I will still be holding each other tightly amongst the carnage. God knows our hearts and he will judge us one day…and we are not afraid.

Tuesday, February 17, 2004 0:58 AM CST

Well it is time for me to weigh in. When Debbie told me she posted our story on the web page I must admit I was a bit taken back, but she said she needed to get it out and the journal has always been a good tool for that kind of release. So here is my take on all this. At the end of World War II, young men returned home and tried to “get back” to a normal life and forget about the horrors of war. For many it was a difficult thing to do and inevitably they gravitated back to each other because they held a common experience, they could understand each others feelings, they shared a common bond. Many formed riding groups and bought up the surplus Harley Davidson’s they had ridden and loved while overseas. These first biker groups where really veterans looking for some comradeship and fun. The craze caught on and as America changed in the late fifties and early sixties so did these groups. They began to get a bad rap as derelicts that were living outside of society’s acceptable standards. For the most part the veterans were gone now, much older and advanced in their lives, families, and careers. A small group of bikers began to receive national attention for there ruff lives and involvement in crime. They were known as the 2%’ers, though they received all of the publicity they represented only a small portion of the riding community. So what does this have to do with anything? Noah was brave and strong, and so beautiful. His experience has helped many children that have transplanted after him, and his story has traveled the world because we wanted to share his struggle. Now things are done differently at Duke; children have a better chance because of what Noah taught the team. But Noah was not their first transplant, he was diagnosed relatively early, his prognoses going in was very bright, and he was at the best place, with the best doctors and nurses, money could buy. So what happen? We ask this question often and no one can answer us. What happen? The problem was that Noah was a 1%er, and in many instances the first. He did not represent the typical Krabbe transplant child. He did not respond to treatment the way years of experience and science predicted he should, he went out side the norms, he was a rebel… but not by choice. Veterans of transplant, who recount their war years may have had similar experiences and loss, but their experiences are not like ours, if they were they would be a member of the 1%er club. Noah’s complications and cause of death are still a mystery to this day, his autopsy has produced no answers other than his body was in distress. There are no signs of infection of virus, the killer for many transplant children, and he did not have graph verses host. No Krabbe child has had mixed cells from stem cell transplant, and the consensus among the doctors has been that the few he had would eventually have be killed off. So we ask why again? Unknown… lethal doses of chemotherapy should have done the job, you made a strong baby, he beat chemotherapy, we were told. We didn’t want him to beat chemotherapy we wanted him to live. We were told several times that our child would die because he was not getting better, and no one, even to this day, can tell us why, it is all speculation. In frustration one day I told a doctor, “Don’t tell me you don’t know, it is your job to figure it out.” but they still could give us no answers. We grieved because we would lose him; than he got better (keeping in mind, better is a relative term, it meant he was off ventilation) only to be followed by another set back, again for unknown reasons, again told we would lose him. For nine months we endured this, but worst of all Noah endured it. We put him through transplant to try and save him and in the end he suffered and died in our arms. This is not about Debbie and I, anyone who truly knows us, would know we would give everything we have to save our child. This is about a child, a child we will not allow to suffer, and than die an agonizing death over a year’s time. I have already heard it; Duke has been successful with infant transplants, look at the children, and look at how well they are doing. I ask you, are they members of the 1% club? If they were, those children would be gone and I am sure their outlook would be different. So before anyone tries to change our minds. If you are not a 1%er, are there are none out there, don’t even think about it. Be thankful you are not in our club of three, reduced now to two. And if our decision troubles you, than walk away, we were glad to know you, and we understand, because when the smoke clears Debbie and I will still be holding each other tightly amongst the carnage. God knows our hearts and he will judge us one day…and we are not afraid.

Thursday, February 12, 2004 6:58 AM CST

We appreciate the support and prayers that we have continued to receive. Despite our best efforts, we have found it difficult to summon words to express how we have felt the last four months. We are trying to survive…put the pieces together as much as possible, to continue to function. I surely hope that I will not regret writing this journal…having to face undue criticism and judgment…but we surely will I am certain. Before you decide to write your disapproving comments…those that choose to, please attempt to keep an open heart and mind…attempt to slip off your shoes and step into ours. I like the old-fashioned saying “If you can’t say something nice, then don’t say anything at all”…it would be much appreciated now.

So I suppose you are wondering what I have begun babbling about. Well, I will dive in with both feet and just get straight to the point. We are now 3 months pregnant…have known for 2 months now…waiting to do the CVS test, a test done similar to an amniocentesis, but earlier along in the pregnancy. Our results returned 3 days ago, showing, without a doubt (99.99999% certainty) that this child is a Krabbe child. We have agonized for weeks leading up to this time, as to what we would do if the child were affected with this fatal illness. We are still uncertain…but leaning towards terminating the pregnancy. As much as we are not for this, we have shared over and over again, the pros and cons of making such a decision…it was not done in haste, and not without a lot of emotional turmoil. And so I begin…words expressing how I feel…they can never do justice to the pain that is within me, but I must get this out in the open.

It is a boy, our second son, who will be lost to this horrific disease…we are truly devastated, but not enough to quit trying…we are not quitters…we never quit with Noah’s transplant fiasco…never…until he let us know he was done. However, at times lately, I am thinking that terminating the pregnancy is the ‘easy way out’, that we are throwing in the towel and being quitters. We are not fighting as hard for this child, as we did with our first…and that isn’t fair, but we don’t want the suffering to be prolonged, and we want to be able to spend the next months trying again…not waiting for delivery of this dying child, only to go through another transplant that may or may not turn out the way we hope. We have agonized over this…have received only one criticism, saying that abortion is like ‘playing God’ and that God gave us this child for a reason…maybe so, but this person has not had to watch their first and only child suffer and deteriorate for his first and only year of life. Noah’s body, his poor little body, took so much insult from transplant. His autopsy showed that his heart was greatly enlarged from being overworked, with the heart valves beginning to give out, his lungs were severely scarred from the continuous aspiration due to his loss of the ability to swallow, gag and cough; and they eventually failed him. His kidneys were severely damaged; on the verge of kidney failure…he required multiple doses of different diuretic drugs just to keep the excess fluid off because his kidneys could not process it out effectively. His liver, also severely damaged and enlarged, making it difficult to break down the chemicals being introduced into his body daily…medicines that were supposed to be healing him…they were only hurting him more. His spleen, also severely enlarged, overworked and damaged…having difficulty doing its function. His brain, not only being destroyed from the progression of his disease, but he had a hemorrhage (bleeding) in one area, and an infarct (dead area of tissue caused by lack of blood flow to the area) in another part of his brain. This was all on the inside…his transplant journey had destroyed his body…he suffered so very, very much…and we could not allow that to happen to our second son. When we arrived at Duke he could see, smile and laugh, suck and eat from a bottle, move both his arms and legs; and by the end he was blind, fed through a surgically placed tube, on a daily medicine regimen that included over 25 different medications given around the clock, he no longer moved his extremities, could not suck or swallow…not even able to gag on his own secretions, they would just flow down into his lungs at free will and he could not do anything. Unbelievable…to imagine that feeling…drowning in your own secretions, choking, but unable to cough them up to clear your airway to breathe. What a terrifying image…and he could do nothing. I watched every minute of this…and cannot relive it again with another child. We just couldn’t do it. It is the matter of choosing the lesser of two evils, deciding what will be the easiest to overcome and cope with…causing the least amount of suffering for everyone. We saw over 12 transplanted children die while we were there, approximately one every 2 ½ to 3 weeks…children that we had come to know and recognize on the bone marrow unit…it affected us greatly…making us pray desperately that our child would not be next…except eventually he was…and the other parents began praying theirs would not be next. True, there were quite a few that made it out alive, some did and came back to die later…some are still out alive and thriving…but they live each day still in fear…it is no way to live. Transplant can be and is a good thing…but it can turn into your worst nightmare if it doesn’t work in your favor…no matter how well intended it is to save lives…it also leaves the door unlocked for death to creep in unexpectedly and steal your child from you…death lurks in the shadows and waits to choose its prize…you just sit and pray that your child is not the soul it is looking for. I wish so much that everyone could understand this…but they cannot, not until they have experienced the same…exactly the same, and that will never be…some may only experience similar events and have something to relate to, but not know exactly how you feel. I wish so much to be honest and open…to be able to express how I feel and why we have come to this decision, without receiving criticism…but it cannot happen. Unfortunately, as humans, sometimes we feel the need to voice our opinions on topics we feel truly strongly about, whether the opinions are based off of morals/religion or logic…but not having the first hand experience to understand the situation, we stand on a soap box that is not grounded with knowledge that comes with personally enduring such experiences. Yet, so many cannot remain silent, be still, just listen and be there…they feel they must speak, and they do.
So why don’t we choose to transplant at birth…it has worked for approximately 10 children. Transplant at birth…somewhat different than our experience…as we walked in the door with Noah having moderate progression of his disease, the second phase symptoms clearly exhibited. He hit just about every bump in the road possible. If there was a 1% chance that his central IV line would pull its way out of one vein into the heart and worm its way up his neck vein toward his brain…it happened. If there was a rare chance that his temporary nasal feeding tube would be misplaced up into his esophagus rather than into his stomach…causing severe vomiting of blood from bleeding ulcers in his esophagus, it happened. If they had never seen a Krabbe kid who did not engraft and lose his transplant…it happened. If they had never seen any child react to a particular chemotherapy medication…it happened, and he almost died that night. If they had very rarely seen a VP shunt fail, it happened…and they had never known it to happen due to the abdominal pressure was higher than the pressure in the brain causing a backflow of the shunt…but still, it happened. If it were possible to rip out of his head the external shunt draining out excess spinal fluid from his brain, it certainly happened, requiring an immediate second surgery in less than 24 hours from the first…this event too was rare. I could continue with all of the “firsts” and “rare” events that took place in Noah’s room on the bone marrow floor. We hit basically every bump in the road…literally. The early transplants at birth have shown success…and that is so encouraging, but not to us…as we have been told that any child of ours could very likely have a failed transplant because genetically Noah’s blood system and bone marrow were much stronger than the lethal doses of chemotherapy, his old Krabbe (no-enzyme making system) system grew back and killed off all of his donor cells…despite our best efforts, his disease progressed anyhow and stole him from us. Thus…we were forewarned when leaving Duke, with Noah’s ashes in hand, that if we returned with another child…transplant may certainly fail again, and we would leave again empty handed. How much more do we keep trying before enough is enough…how much more suffering must we undergo until mercy is shown. Must we be persecuted because our human weakness won’t allow us to take on this challenging road again…a path down which we not only lost our child, but watched suffer a most horrible, prolonged deterioration leading to his demise.
No one, absolutely no one, can ensure that this child we now carry, if transplanted, would survive it. No one can promise us that the transplant won’t fail, as it did for Noah, the first Krabbe kid to have a failed transplant. No one can be certain that this child would not suffer severe complications. There are no guarantees…and what we want is a CVS test that can at least guarantee us a child free of Krabbe. God may not agree with our decision to abort this pregnancy, and maybe He had more plans for us as parents, to attempt transplant again…but I also know that God offers forgiveness…no matter what the transgression, to those who humbly come before Him and ask. I don’t purposely do wrong, knowing that I will be forgiven, but He knows my heart…and He loves me still. The Bible says as one of the Ten Commandments, “Thou shall not kill”…but my husband, a soldier in the US Army, sent off to a war with a gun or rifle in hand…caught in a firefight with the enemy…and his enemy is shooting to kill him…is he expected to lay down his arms and walk away…no…he will defend his life, even if his enemy is killed. This is done daily, especially over the last couple of years…and these people are not punished. They are given medals of honor, sacrificing their lives for freedom in attempt to eventually bring about some form of peace between warring states . In like manner, we are faced with Krabbe, the enemy which will surely kill our child, but only after causing great prolonged suffering…there is no question…are we to just sit idly by and turn our cheek…and if we choose not to take this path, are we to be condemned for it? We are doing what we believe is best, and God knows this…I truly believe this. We are not trying to destroy a child that is not wanted…we want a child, we wanted THIS child so very, very much, but we don’t want to prolong the suffering of a child that will surely die to satisfy our selfish need to be parents. I am sorry to ramble on so, my point was taken a while ago I’m sure and this excerptation was most likely not needed…but I am so very, very tired of being judged by other humans…sinners just as I…”ye without sin, cast the first stone”…there is no one, except God and His beloved son. I say all this, I suppose, simply to reach out for support and understanding…not criticism and questioning of our moral character. Most couples do not ask first to have children…they just do it, almost expecting it to happen…as if it is their right. We have not only asked, we have begged, pleaded and prayed…why are we so different from them that the children we receive must die shortly after they are given to us…what lesson are we to learn that they do not need to grasp? These questions will remain unanswered until I am before my Maker…I will ALWAYS wonder why, have regrets, carry some guilt and grieve the losses…always. I will likely wonder at times, ‘what if’…again, this falls into the category of regrets…but I will know that I did the best I could at the time…my best intentions were meant, no matter how the situation appears from the outside.
Thank you for listening to me carry on WAY too long…I have so much emotion pent up inside that I feel like I will explode at times. My heart remains heavy, my shoulders laden with so much to carry, guilt and pain placed partially by my own accord…no more is needed by my fellow man. Tomorrow I think I will rest, giving it to God, praying he will see us through yet another terrible storm.

Thursday, January 22, 2004 12:36 AM CST

This is a copy of a letter sent out by Jim and Jill Kelly to all of the families associated with Hunter's Hope, regarding the annual luminary service held on Hunter's birthday, in New York. By sending a minimal donation of $5.00, a luminary may be purchased for the candlelight event, with a message attached in honor or in memory of someone...i.e. we will be purchasing a candle, a.k.a. luminary, in memory of Noah to be lit during the event. We wanted to give family and friends the opportunity to do the same. Again, proceeds raised by Hunter's Hope go to help fund research for Krabbe Disease and other leukodystrophies to help save the lives of our kids, and the families the heartache of watching their kids suffer and die. Thank you.

Dear Friends,

This coming year, on February 14th, we will once again be celebrating Hunter’s Day of Hope for Children at Chestnut Ridge Park in Orchard Park, NY. It is a true celebration of children - adhering to our mission of encouraging parents to love and appreciate their children more and thank God for such a gift of life.

At this time, we will be lighting luminaries upon the sledding hill of the park. Our goal is to be able to spell out “Hunter’s Day of Hope 2004” in luminaries. Last year was so beautiful to see Hunter’s Hope spelled out on the hill as it was visible from great distances!

Please help us and be a part of our goal. With a donation of $5 or more, a luminary will be lit in memory or in honor of someone you love. All you have to do is fill out the slip below, tear it off, and send it along with your donation to the Hunter’s Hope office. With your contribution, and contributions from your friends, family, and others, we will be certain to light up the night!

Please share this form to all of your family and friends. If you need more copies, you can print this form off our website at www.huntershope.org.

Thank you for choosing to light your heart, and ours, with hope. May God bless you!

With hope,

Jill and Jim Kelly
Founders Hunter’s Hope Foundation
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Please fold/tear along line and postmark by January 31, 2004
Please mail to: Hunter’s Hope Foundation, P.O. Box 643, Orchard Park, NY 14127

Your Name ___________________________________________________________

Address __________________________________________________________________________________

Please accept this donation of $___________ to the Hunter’s Hope Foundation.
I would like the luminary to read as follows:

Monday, January 12, 2004 11:30 AM CST

We are on a long trail now and voices call out from dark recesses on either side with unseen faces. Ahead is a terribly large and dark mountain with billowing grey and black clouds, pouring down the side and crashing at the base, rolling like thunder towards us. We can only lean into this misty undulating mass, and feel the cold spray on our faces, as we continue to press on. It would be easy to sit at the side of the trail and rest, to let the storm pass us by, to look at the mountain from a distance and wonder at it’s size and power, but we can’t…we must press on. Somewhere ahead, we can hear the cry of a child, it calls out to us in all the confusion and uncertainty. We must stick to the trail and face the mountain, fight through the haze and suffer the bitter cold because, there is a child waiting to be held and comforted in the storm. Like spring waiting for the winter to pass to bring life anew again, we must weather the worst, hoping to conquer the mountain and comfort the child, to pass through spring to summer and leave the winter behind us. This is our daily struggle; hope is all that sustains us now, our shelter from the storm.

Tuesday, December 23, 2003 3:27 AM CST

What do we want for Christmas? I will tell you nothing, nothing but the chance to have a healthy baby. You see we loved being parents; we lived to be Noah’s Mom and Dad. We can not replace him, and if God blesses us with a child than it is Noah’s little brother or sister, not a replacement. No one could ever replace a child as beautiful and perfect as Noah. So if you want to do something for us for Christmas than take a moment and pray. Pray that God will give us a child free of illness. That is all we want, to be parents again. To hold a child in our arms and love him or her like there is no tomorrow. There is no greater gift on earth than a child. We pray night and day for this. This Christmas may God give all the families and children that suffer so much some peace, this is my hope. Thank you for all your support and prayers…may peace be with you all.

Thursday, December 18, 2003 4:25 AM CST

I wrote all of NoahÂ’s journals the last week of his life to include his good by and then I was silent, until I posted the letter I will be sending to congress. For those of you who have continued to check up on us I am sure you saw DebbieÂ’s last entry. I am brought back to thisÂ… Great men overcome insurmountable obstacles to become great. I will not tell you now, that I feel great, nor am I able to tackle even the smallest of obstacles. So I wonderedÂ…am I week, have I failed. I am a history major and so I looked to history for an answer. Abraham Lincoln lost his son, and when he did, history noted the change in him. The government went on, and the civil war continued, but from that day forward he was a changed man. He spent many hours isolated, depressed and alone, in his private journals he even talked of suicide. Fortunately, he surrounded himself with people that knew his vision and made sure his wishes were carried out. The nation and the war went on, with minimal decisions on his part for a period of almost a year. Many historians will say he was never the same, nor his wife. But no one will ague that he was not great. You see the nation understoodÂ…the people showed him compassion, compassion for his loss, he was allowed to grieve in his own way, because he was the President.
We will grieve in our own way also. It is difficult in our house hold. If it was not for our strong marriage I donÂ’t know how we would have survived. Our house is silent. It is hard to comfort someone when you are uncomforted. How can you listen, when inside you hope to be listened to also? You must understand that every minute with Noah was a blessing but also a nightmare. We went for a year without a nights sleep. Sometimes we went days without rest or food. We hoped, we prayed, we begged. We only wanted to save our son. No matter what we did, it ended in tragedy. There were no breaks, only another challenge to face. For him we would have given our livesÂ…gladly.
No one really knows the end. I will tell you now because it haunts me day and night. We rolled with him down the hallway from the PICU to the bone marrow floor. I was awash with emotion, focused on the monitors at the end of his bed, his O2 well below 50%. Debbie buckled, and fell crying, I lifted her up but never lost site of the monitor. Noah looked grey, and pale. We had problems rolling him in to the unit, Doctor Kurtzberg forced the bed into position so we could get him to room 5206Â…The room next to the one he had spent so much time in 5205. The anxiety mounted. I acutely felt like he would pull through, and surprise them all one again. But when we go him into the room, I soon realized that there was nothing anyone could do. They handed him to us, and Debbie cradled him as I supported his head. There was a lot of people in the room, and our Pastor asked for prayer. I asked everyone to leaveÂ…there was no time for prayer, no words could be said, and we just wanted to hold our son in peace. But there was no peaceÂ…Noah exhaledÂ…it was raspy. We held him tight, telling him we loved him, the tears rolling down our faces. He clutched DebbieÂ’s hand and opened his eyes, looking at me. I stoked his head and pleaded with him, go to sleepÂ…please go to sleep. I felt as though I was going to explode. He turned his head and shut his eyes. He turned purple, and his face grimaced. We continued to hold and rock him, telling him we loved him. I called for Doctor Kurtzberg, afraid he was suffering. She pulled the stethoscope from the wall and listened to his heart, and told us it was still beating. We watched him turn grey, he continuing to breathe out in a gruff toneÂ…and then he was gone. The room was spinning; I could not focus on anything. Debbie would not let him go, she held him tightly. Hours later as we left the floor, I looked over my shoulder as the door to room 5206 closed. There was my son, dead. I will never forget that moment. I can see the door closing, while I tried to capture that last glimpse of him. Not wanting to leave him in that silent room alone. These memories haunt us every day, I see this moment in time over and over again. There is no getting over it. We can only ask that everyone understands. And if at times we seem angry, or distant, look back at our story and try to realize our pain.

Tuesday, December 16, 2003 5:25 AM CST

This is a copy of a message I sent to a reader of Noah's web page in hopes of helping her to better understand how I feel right now. Maybe it will be helpful to more than just her, so that is why I decided to post it here.

My comments on Angel Reese's page were for his grieving mother, Shawn,...yes, from one grieving mother to another. I am sorry if my candor offended you, but Greg and I have always been honest and candid with our thoughts and feelings...no changes now. Noah's journal, our journal, was simply created and recorded on our Microsoft Word...and saved each day as our way to record our personal thoughts and feelings about what we were enduring. Originally we had written a separate entry for Noah's Caring Bridge page...and it was so that our circle of close family and friends would have a way to keep up with Noah's status while we were away at Duke, because it was impossible to contact each one daily. We were always very open and honest about what was happening with Noah, his status, his treatments and above all, what we thought and felt about the events at the time. Unfortunately, in doing so, we opened ourselves up for criticism, those who wanted to question why we thought or felt a certain way, or judged us for feeling or thinking a certain way. Over time, unbeknownst to our expectations, the amount of readers soared into the hundreds and if I may be so bold to say, maybe even into the low thousands. These were strangers, who logged onto their computers to read an emotionally moving story. I guess it could be compared to those who sit down each afternoon to watch their soap operas...to follow the continuing saga and tragedy that was shown before them for that brief hour. Afterwards they could turn it off, and pick up back where they left off with their day. Likewise, the many could read the daily journal, peruse the guestbook to read other peoples messages to us, opt to leave one of their own, glance at the photos and log off, to return to what they were doing. Some of these strangers returned on many occasions, and many left messages that were positive and encouraging...in attempts to cheer us on. We did enjoy reading them, and usually made our practice of logging on to do so daily, after posting a new journal. Occasionally when we would miss a few days of journals or didn't post new pictures for a week or longer, we would receive messages and emails inquiring about when we would update...like an addictive novel reader probing the author for his next edition. My point in carrying on is that it seems of these many individuals, who were kind enough to show support, and were genuinely concerned about our son, they began to feel as though they knew us so well and were part of our lives, when really...it was all one-sided, and we knew nothing about them...and relationships/friendships cannot be built one-sided, not true ones anyhow. We appreciated the messages left in the guest book, those who actually donated money to help us out, those that sent Noah some toys/gifts, those that made donations to Hunter's Hope in his name for Krabbe research. We greatly appreciated the prayers said on Noah's behalf. If you have ever lost a child, then you easily recognize that it is so very, very different from losing a friend, a parent, a grandparent or aunt. Extremely different, and if you haven't, then I do not fault you for not understanding where I am coming from. You never heard about us and our lives before Duke, you don't know what hell we went through together in the three and half months before he arrived at Duke. They were not "good times", as Noah was affected in utero, and showed signs from the moment he was born, crying 20 hours a day inconsolably...total misery for us all. I saw him smile two times, never heard him laugh, but saw many months of him suffering terribly. There is so much more, but I won't bother you with it. I do want to say, that I am blessed to have a great friend that I work with in the PICU, who has three beautiful children. She understands the fact that a part of grieving is anger, pain, deep sadness, and emotional anquish...that the person who is enduring it is the only one who can work through the grief and must do so at their own pace. Some work through grief quickly, others grieve for a lifetime. She recognizes that she, as a friend, can help facilitate the process and help by just being there, nothing more. She knows that her words are useless, but her ears and her shoulders are priceless. She takes no offense of my harsh words, full of anger or sadness, she knows that they are a release of pent up grief and emotion, that must be released in order for me to survive; and that they, like the high tide, will pass and allow me to rise above the drowning waters and paddle myself forward against the waves of depression and anger, in an effort to reach dry land. These words are not about her, they are simply ventilation of grief. So, my entry into Angel Reese's guest book, written to my friend Shawn about her beautiful son Reese, who I was so very blessed to see, whose hand I was able to hold and face I was able to caress in his last days of life, was simply my ventilation of grief, letting her know that her expression of words and thoughts about her grief, were quite similar to my own...nothing more. If you take offense to them, sorry, they were not about you or to you personally. They were simply said on one of my bad days, and I am allotted some I think, as everyone seems to have them, even those who aren't grieving; but I am not going to pretend that I don't feel or think those things at times and am not going to stop openly speaking now after months of candidly writing my thoughts and feelings for everyone to read. I am sorry if these words are not as exciting a story as Noah's suffering; but they are of my suffering. We are working through our grief daily...and are not at a point in time to simply celebrate Noah's short life, his bravery, or the 'good times'...maybe one day we will be, but that time is not now.
I am not asking for you to write the "right" words, and please, if you don't take anything else from this message, please remember, I don't expect you to have any words to help me...there are none. I said that already months ago in a journal, there are not any words that will bring back the son that I so desparately cry over losing each day, who never leaves my mind, whose entire short life and the events surrounding it replay in my mind night and day. I never expected anyone who left messages in Noah's guest book, whether it be someone I knew personally, or a stranger whom I allowed to know me personally, to know what were the "right" words to say. I don't even know. I can't tell you. I can tell you that if you give me some aspirin, it will help my headache, but I cannot tell you what will help my heartache. Believe me, if I knew, I would have already persued it by now, but I am finding that it is a good, understanding friend (a set of ears, a closed mouth, and a person who wears a rubber suit allowing all things said to bounce off, but still continue to support and love me) who has been of great relief, and I am thankful to God for at least allowing me this.

Again, I don't know you and I am sorry if you are offended by my message to Shawn, but I don't apologize for what I said to her, as it was my way of coping on a bad grief day...or as we call it in our family, I was having a "Noah Day". I recognize your messages left in Noah's guest book only by your name, and I know that you have left us a lot of messages to show your support. I am very thankful for them, but they are not as helpful right now to me as just being understanding and allowing me to curse, spit, rant and rave about how pissed off and hurt I am at God, probability, genetics, or life in general. You are correct in what you say...Noah was brave, strong, we were lucky to have him, he touched so many lives, he gave a good fight, and we should try to focus on the "good" times, but I am not at a point yet where I can do that...and that is why I wrote what I did. Because it is frustrating to hear these things continuously from people who care and are just trying to be supportive and knowing that they are true statements, but not feeling that way; or being frustrated that I know I should feel that way, but I don't...I am working my way to get to that point, but it will only come in time and I am not there yet. I hope that this in some small way will help you to understand where I was coming from. It is the best explanation that I can supply right now.

I hope that this was in some way helpful...and if not, well, then I guess it wasn't. What I have learned is that everyone deals with grief, stress, and loss in their own way, and that not any one way is the 'right' way; some are probably better than others, meaning productive vs. destructive, but there are no rules to follow or any right way to work through it...you just have to do what works and try to do your best at getting through it. I learned in school a while ago that the five stages of grief (as theorized by Elizabeth Kubler-Ross) are Denial, Anger, Bargaining, Depression, and Acceptance; but more importantly that everyone goes through them in a different order/pattern and at their own pace. However, she never gave any guidelines that I can recall to the friends of those who are grieving as ways that they can be of help...or maybe it was that I didn't have to remember it for the final exam. Maybe someday some genius will develop the guidelines that will help us all understand and know the "right" words to say or the "right" things to do, but until then we are left to try and figure it out by ourselves with trial and error, trying to do only what we think is most helpful and, of course, "right".

Tuesday, December 2, 2003 5:17 PM CST

Below is a letter I plan to send to try and help get legislation passed to provide for newborn screening. I thought I would share it with all of you. Noah's memory deserves this and so much more. Feel free to copy or alter it to send to your congress person if you would like to help.

Peace.....

2 December 2003

From: Sergeant First Class Gregory S. Prichard
392 Akana Place, #102
Honolulu, HI 96818
Phone (808) 836-8055

To: Senator/Representative
(I will look up mailing addresses and names later)

Senator/Representative (Name)

I am writing you in the hopes that you will support current legislation being introduced in the (House or Senate?) by (?) regarding mandatory screening for newborns. This legislation is important to my family and many others across America. Our story may help you to understand why I write to you for support of this bill.
My wife and I waited six years to have a child. We wanted to make sure we were financially and emotionally ready to provide for a new member of the family. When our son came it was the best day of our lives, we were thankful to have such a healthy and beautiful child, but within weeks, we began to sense that something was not right. Our son cried, he cried sometimes up to twenty hour a day. We repeatedly took him to the doctors, and were continually sent home. We were told that he had colic, or that it was just his temperament. Until one night, in the emergency room, a doctor finally listened to us and ran a full set of labs. The tests came back negative except for some elevated protein in his spinal fluid. This puzzled the doctors, since he showed no signs of infection. He was placed in the PICU, and more tests were conducted. Several days later, we were told that our three-month-old son had Krabbe, a genetic metabolic illness, and that he would probably die within twelve to thirteen months. Our world came to an end.
We were informed that there was no cure, but after a day of research we discovered that Duke University Medical Center was successfully transplanting children with his disease and other metabolic illnesses through unrelated umbilical cord stem cell transplant. Within days we were in North Carolina, Duke was our only hope to save our son.
We stayed at Duke for almost ten months while he underwent transplant. He had many complications, and we made several trips to the PICU. After months of struggle and endless days and nights fighting to save our son, we lost him on 7 October 2003 a few weeks after his first birthday.
We lost our son because his illness had progressed too far before we could get him treatment. Our story is one of thousands across America. If the hospital would have screened for this illness at birth, he could have been transplanted earlier, and might still be with us today. Mandatory newborn screening would have given my son a chance at a normal life.
Our nation is full of bountiful resources; they stretch from sea to sea, from our industry to our farms, national parks, and oil reserves, the list goes on and on. We protect these resources because we know they will secure a future for America. My argument to you today is that our most precious resource, our children, must also be protected. Our children are the future of America; they are the next presidents and politicians, scientists and doctors, inventors and scholars. Mandatory newborn testing is a low cost way to protect the future of America and will save countless lives.
I have enclosed a picture of my son, we will never know what he would have become and how he would of impacted this great nation. We can only share his story in the hopes that future children can be saved. I ask you, can America afford not to protect its children? I encourage you to consider sponsorship of this bill, so that we can preserve our most precious national resource, our children.

Respectfully,

Sergeant First Class Gregory S. Prichard
United States Army

If you would like to learn more about Noah’s journey we created a web page for him that contains our journal entries while at Duke? Click on 'previous journals' to view our experience. The web page is www.caringbridge.org/hi/noah .

Sunday, November 30, 2003 7:50 AM CST

Sunday, November 30, 2003

It’s been awhile since our last entry, there remains not much left to this story. We have both returned to work and have attempted to recuperate what were once our normal lives, before Krabbe was knowingly brought into our lives. There remains no official date for a memorial service, but we are looking more towards the first week in January (possibly). We ask for your prayers of peace and healing for the families of Manny, Princess Madi, and Tommy Bennett, who have now passed on to reside with the rest of our little angels, waiting for our arrival. We also ask that you would continue to remember those children and their families who continue to face the battles of fighting these terminal illnesses, and those undergoing transplant. We don’t come to Noah’s site anymore, except once in a very blue moon, but I skimmed through some of the guest book and want to say thank you again for your prayers and support. We pray that you all have a wonderful holiday season. God bless.

Thursday, October 30, 2003 6:53 PM CST

Entry 194—October 30, 2003

We made it home safely, but it was a VERY LONG trip home…getting up at 0630 in the morning and arriving home 18 hours later. We appreciate all of the cards, letters, emails and messages left for us upon our return home. We are so grateful for all of the encouragement and prayers. We have begun the process of packing up Noah’s nursery…what a job. We have been doing some house repairs and cleaning to get things back to what they used to be, somewhat. Our vehicles, parked for over 10 months, are now running fine and are legal again…but rather costly to get them taken care of. However, things are pulling together somewhat and we are attempting to reorganize our lives…if it is at all possible. It is like trying to put back together a puzzle that has been torn apart, but now not all of the pieces are there, so it is left unfinished…to be left undone. So we have to cut out new ones and try to paste them in the empty spaces to complete the picture, but it still doesn’t look the same…and our eyes cannot be fooled. After losing our dog Yzerman in June, a significant part of our family, and then Noah…the crux of our family, the house has been extremely quiet. So, we adopted a new member, Fritz, an 11-week-old German shepherd, and are attempting to acclimate him to our home. He has certainly brought some excitement and has definitely kept us very busy trying to keep his curiosity at bay. Noah remains here with us, not only in spirit, but his ashes are snuggly kept inside a cuddly teddy bear in our den. He will be with us always and we can freely pick up his teddy bear and cuddle and hug him when we are feeling down…it is so very nice and we couldn’t imagine it any different. We have about 10 more days before we will both be returning to work, Greg back to his M.P. unit in the Army, and I, back to the PICU at Kapi’olani. I believe that I can speak for both of us in saying that we are a bit anxious about stepping back onto that moving train, but also in need of some structure and routine to keep us focused. Not to return to ‘life’ would be a great dishonor and disservice to our beautiful son…who never gave up on life, but rather his body gave out on him. He fought up to the very last heartbeat…and we are going forth to live our lives, and not allow death to drag us into a pit of sorrow and depression. We will make him proud.
We have not been on the internet since the night before we left Duke, so I have not managed to check up on the other kids that remain there with their families, fighting through the pain and suffering, to survive; but they have not been far from our minds. We ask that you will continue to remember them in your prayers as well. There are so many that need some special prayers. We will be planning a special memorial service in Noah’s honor soon, to be held here in Hawaii, and we will announce the date and time as soon as we are aware for those that can and want to attend.
We would like to offer our sincerest thanks to those who made special donations to Hunter's Hope in Noah's honor. We are so very, very grateful. Please know that your contribution will go to help further research about Krabbe disease, in hopes of finding a cure and saving the lives of so many innocent children just like Noah. Thank you again for the thoughts and prayers. God bless.

Monday, October 20, 2003 9:52 PM CDT

Entry 193—October 20, 2003

Our last night here in North Carolina after spending over 10 months here and we have so many emotions overflowing within us right now. We are anxious and eager to return home, to get back to a ‘normal’ routine and life, to continue to work through our grief and learn to live life without Noah, …but then that means closing this chapter of our lives and I am not sure that I am exactly ready yet. Our weekend spent with fellow Krabbe and transplant families and friends was indeed bittersweet, but it was nice to see so many of them before we left. Kelly graciously hosted the event for us (THANK YOU KELLY) and we released 35 beautiful white balloons in honor of Noah. It was absolutely breathtaking…the sunset illuminating the pearl white balloons as they floated up into the heavens towards our angel baby Noah. It turned out to be a very nice evening spent with so many wonderful people that we have come to care so much about.
We are glad to finally be returning home, but we will miss all of the people that we have come to know as family here at Duke. We would like to thank everyone, far too numerous to mention by name. Your uplifting encouragement, prayers, unwavering support, and kind generosity helped carry us through some of the toughest times and experiences in our lives. God bless and keep you. Peace be with you…

Friday, October 17, 2003 10:28 AM CDT

Entry 192—October 17, 2003

It seems so weird not to have anything to write about anymore. I don’t want to write about how much this sucks and how much we miss him…that should be the obvious to anyone and should not need an announcement of declaration. I guess as always we can just share what has been going on…not much. We have been boxing up our stuff that we have accumulated this year during our stay, and taking trips to the post office. It is amazing how expensive postage is, and it is too bad that we don’t have the luxury of just renting a U-Haul. We are getting it sent off though, and should only have a couple more trips.
Yesterday I was able to go to lunch with Kelly (angel David’s mommy and Hunter’s Hope family support representative) and Lori (angel Megan’s and Degan’s mommy), and then we went shopping for a bit. It was a chance to escape for a while and try to get back onto the rapid expressway called ‘life goes on…even when yours is at a standstill’. I think sometimes that hurts almost as much when your entire world has come to a sudden stop, and that the world around you continues to move along…and when you have to try and get back into that fast-paced world, it all seems too weird and crazy to have once been the ‘norm’…and even weirder to have one person less in the family that you traveled down this road of life with.
The sympathy cards and messages taper off and then it seems you are once again experiencing that isolated feeling, the feeling I had when we were still at home and told that Noah had Krabbe disease and would die a horrible death within the year; …and the isolation felt when Noah’s donor cell engraftment failed and was the first cord blood transplant for his disease to have this outcome (and the proverbial “we don’t know why this is happening”)…the same isolated feeling when we were walking across the long dark hallway last Monday night knowing that there was no turning back and in a matter of minutes he would be gone from us forever. In most all of these situations there were in fact people present, but it was not their lives that were being turned totally upside down. It was not their child that was being diagnosed with a death sentence or who had to endure painful treatments only to be told that they didn’t work as they should have; and it wasn’t their child that would be taking his last breath in just a matter of minutes, to be gone from them and their embrace for a lifetime…left only a cold shell to hold that had been touched by death. Well, there are no words to make it all better, and I suppose that sums it all up…there simply are no words and the pain will be there forever in some shape or form. Just as the old ‘When you fall off the horse…’ analogy goes, I suppose the only way to pick up the pace of our lives now is to just ease our way back in to the flow of life, taking with us all that we have learned from our hazardous detour.
We know that we were never truly isolated…we know God was there…somewhere, but he didn’t really make himself known to me until it was over. We had plenty of supportive people here at Duke; the staff, friends and other families alike, and support from the religious readers of Noah’s web page. We have declared our thanks to God for these blessings, and we have concluded most all of our journals with expressions of our gratitude for your encouragement and support…and we truly are grateful. I suppose that we are now in a position that no one else can move us from except us…we must be the ones to stand on our own two feet and begin to make the steps forward. This is where we are at, and as is the case with a lot of things, it is the ‘getting started’ part that seems to be the hardest, but it will come with time I suppose.
Our final meeting with Dr. K is this evening, where we will once again express our gratitude to her for all of the wonderful and amazing work that she does for children and has done for us. At the time of Noah’s diagnosis, she was the only one that would transplant him and we are so very grateful for the opportunity granted to us by her, to give him a second chance at life. She offers those second chances to a lot of kids, what a saint she truly is. This weekend we will have a farewell gathering with all of the Krabbe transplant families here at Duke and a few other special people that we have become close with during our stay. This will be very bittersweet for us, but it is another one of those ‘steps’ that we must take in order to keep moving forward. Thank you to those who stopped by to wish Dylan the best on his transplant day! Please continue to do so, as I know it means a lot to them as it did to us. Please also keep Daryn, Ashleigh, Elijah, Degan, and all of the transplant kids in your sincerest prayers for healing. God bless.

Wednesday, October 15, 2003 9:52 AM CDT

Entry 191—October 15, 2003

We have been approached several times with sincere requests from family, friends and strangers alike, asking what can be done for us during this difficult time. I guess I must first comment on how awestruck we are that Noah managed to touch so many lives in his very short 386 days upon this earth. We are humbled by the impact that he made on this world in such a short time, but this impression reaches beyond the public and Caring Bridge community. The doctors and medical/nursing staff that worked with him have been able to learn so much from him, and we are hoping that since allowing them further research of his body that they will be able to discover a substantial amount of knowledge about Krabbe, other metabolic diseases, and other children who may have a mix of donor and host cells from transplant, in hopes of saving the lives of dying children that follow this path. We are so very proud of him, a sweet and beautiful little boy, born to us, but just dying to be an angel. An angel whose impact goes beyond what we could have ever fathomed. We vowed to do our best, to honor his sweet little life with each day that we are blessed to live, to share about love, faith, hope and compassion; but to also share about Krabbe disease in hopes of saving another life and sparing a family the agony of this horrific tragedy. So for those who have graciously and kindly offered your assistance to us, we ask that you join Noah in his mission to touch other lives, making a positive impact, and to share with everyone you know and don’t know, about Krabbe disease. We ask that in lieu of flowers and such, that donations be made to Hunter’s Hope in Noah’s honor. For those who may not know about this incredible foundation, it was set up by the parent’s of Hunter Kelly, Jim and Jill Kelly, in hopes of educating everyone about this disease, for helping to fund research for treatment of Krabbe and other leukodystrophies (such as ALD & MLD), and to lobby for passing of legislature that would require prenatal testing of this disease (to add it along with the large list of others that are already tested for). There is a web link to the Hunter’s Hope home page down below. If you feel the need to do more, then there is always room for more Hunter’s Hope volunteers! We want to say Thank You, again, for the tremendous outpouring of support, encouragement and prayers that you have blessed us with during this difficult time. You have touched our hearts as well, and we will be forever better for it.

We want to mention a need for prayers for little Dylan, Noah’s Krabbe buddy, who is now on the PBMTU and will receive his transplant today!!! Godspeed little man, and we will be praying for your healing Dylan! Grow (donor) Cells Grow!!! Please stop in to visit his web page and offer your support. This family is from Holland, a very, very far distance from their friends and family, and could use the extra support and prayers that you all have graciously shown to us. God bless them.
www.caringbridge.org/europe/dylan

I know that there are so many more very sick and dying children that need prayers just as much, far too many to mention them all by name here, but please consider them all in prayer. Thank you again. God bless.

Sunday, October 12, 2003 9:22 PM CDT

Entry 190—October 12, 2003

[Noah, I am sorry that mommy is wasting time to do this, but I cannot go without addressing this person’s comments about being disgusted to see your beautiful angelic body, free of breathing and feeding tubes, wires, electrodes, drains, and IV tubings. Mommy and daddy love you so very, very much and will do our best to honor your short, beautiful life with every breath we take, but mommy cannot let this one go without responding. We cannot wait to see you again…you will be without pain & suffering, able to walk and talk to us. We are certain that you are having a wonderful time with Yzerman and Max…remember to tell Max to look both ways before crossing the street for cousin Logan. We love and miss you sweetie!]

I cannot let this one go, I just can’t. First, let me say that I know that Angie did not write that message, because that is sweet little Jordan’s auntie and I do not believe that she would write such an insensitive comment. I believe it is the same “debbie” person that has been signing negative messages to different Caring Bridge web pages for a few months now, and had even signed one to ours a few months ago about how us posting info about Noah’s donation accounts set up by his auntie and gramma was showing how greedy we are, and that our child was going to grow up to be a beggar like his parents. Well, ‘debbie’, our son is not going to grow up with his parents, because he is dead, but is surely alive in Heaven where he awaits us to join him someday. And if I remember correctly, how did Jesus treat the beggars that he came across in his life here on earth…I believe he showed compassion upon the needy and sick. Ahhh, compassion, …ever heard of it????

This web page was not started for your benefit ‘debbie, angie, or whoever you are’. In fact, you would be doing us all a service by not returning to it. That would be one kind gesture we would all appreciate you for doing. Noah’s Rainbow of Hope page was created to let our friends and family have a way to check in on his progress throughout his transplant journey. Over time, our friends, family, and other sick children and their families referred his web page to others, so that we might receive more support and encouragement, as our journey only got tougher and we surely needed the extra encouragement and prayers. The photos that we post with the journals are strictly to help complete the ‘picture’ that the journal attempts to paint…and for those who have not seen an EVD, central line, EEG, or a ventilator, the photos of them are helpful to see for a better understanding of what we are seeing in person each day. For our family and friends who so desparately wanted to share in the pain and heartache of our journey but could not be with us, this allowed them to see some of what we were living each day at Noah’s bedside. I suppose if you are that type of person that would rather see a baby on an oscillator or ventilator (life support) with a few dozen tubes, wires, electrodes, etc. then we could email you some…we have far too many for our taste. However, for those family and friends that could not be here with us in Noah’s final hours, and their last mental picture of Noah was on life support with all of those tubes and wires, then seeing him bathed, groomed and dressed, looking absolutely, angelically perfect is what they need to find some closure. They need to see with their eyes, so they can tell their breaking hearts, that Noah is now at peace, looking more beautiful than ever, and they can find some rest in knowing that he is now an angel. They can be at peace knowing that Noah was surrounded with the love and support and care that was due him in his final hours of life on this earth. His loving family and friends need that…and if you don’t then that is fine. Again, this page was not created for you. I am so very sorry for you, for you missed being in the presence of an angel! We will be praying for you, that you will find love, learn to love yourself so that you can love others. I want to ask that you will first listen and think before you open your mouth to speak. “Listen without defending, speak without offending”. I also want to ask that you will no longer visit Jordan’s site or any of the other Caring Bridge web pages that have been set up for our sick and dying children. It has been made obvious that your ‘encouragement’ is destructive and not wanted by those of us who are in need of genuine love, compassion, encouragement, support and prayers during our darkest hours of watching our children die and then grieving their loss. Enough said, I will no longer waste my breath or thoughts on you again, except to say a prayer for your soul. God bless you with a better understanding of the pain and suffering that we have been through. May He bring some compassion into your life.

As for the both of us, we remain in NC for about one more week, trying to further pack and ship stuff back home. It is amazing how much a person can accumulate over 10 months away from home. I wish U-Haul flew! We are managing our grief, some days are worse than others, but we are not doing too badly. Noah is no longer suffering and we have found peace in knowing he is now without pain, without any needs for medical treatment, oxygen, tube feedings, IV infusions, or ventilators. He is still loved just as much if not more, and will be missed so very much by so many people. We thank you so much for the tremendous support and prayers. We truly appreciate your uplifting words, poems, and encouragement during this very difficult time. God bless you all for your kindness.

Thursday, October 9, 2003 10:37 PM CDT

Entry 189—October 9, 2003

Like empty bottles setting adrift in the ocean’s current, we sit in the midst of our grief, now with empty arms and drained of emotion…drifting along numbly trying to feel our way through this boundless ocean of pain that surrounds us. As parents, what do we do now? We have no one to ‘parent’. Sure we have memories of Noah’s one year of life…days, weeks and months spent in a hospital room with a very sick child, memories of hours, upon hours spent praying desperately to God to answer our prayers for healing. We are grateful for our time with Noah, for we know how valuable life is and the need to savor every moment. We did, …but it doesn’t take away the pain. So he is in a better place, but I felt as if my arms weren’t that bad of a spot for him to be…but that would have meant he’d still be suffering, and I know that this is not what we would have wanted for him. This is the only thing that I can find in all of this chaos to bring me a simple ounce of peace of mind, and I am desperately clinging to it to get me through. We are in the middle of making his ‘arrangements’ and are packing to return home soon to Hawaii with our son, but in a box instead of his carseat. We will never understand why God chose this plan for us, but we have no other choice but to except it and try to make something of what is left of our lives. Thank you for your support, prayers, donations, encouragement, guest book entries, and gifts for Noah over this last year. We could never express enough gratitude for the kindness that has been shown to us and we are forever grateful. We will never forget those who were so instrumental in Noah’s care and treatment, …the doctors, nurses, bone marrow staff, and to Kelly & Hunter’s Hope. We ask that you will continue to remember the other families that are going through the transplant journey, and the many, many others that will follow this path in the future; that they may all find the strength and courage that they need to get through the tougher times. God bless.
Noah, mommy and daddy love you so much sweet angel.

Tuesday, October 7, 2003 3:44 AM CDT

1205AM, 7 October 2003,

We have reached the end of the rainbow, it is a cold and desolate place and in our hearts is only sorrow and tears. Behold this beautiful jar of clay now broken before us. He fought so hard, and we fought along with him. Day in and day out we struggled as a family and prayed for mercy. The days became months and with every passing hour Noah became weaker, until there was nothing left, and God called him home. This beautiful gift we were given was snatched from us, and we are left to ask why? I had a dream, and in it was my son, walking, dragging his Elmo, his blue eyes open and beaming with life, a smile on his face, seen only a few times, dimples and all. This dream was fleeting, a sign of the times to come, a time when our boy would be taken from us. We were made whole in marriage but now we are halved, we had the joy of our son, and now we are left…desolate and barren. This is not an easy task, to write your child’s goodbye to the world, after all aren’t they meant to write ours? But for all the people who followed his journey it is necessary, they deserve to know the end. We grasped our son tightly to the finish, bathed him and kissed him, said our goodbyes, and then finally…had to let him go. We had so many hopes for him, we could see him engrafting and growing up, we looked forward to working with him and witnessing his improvements, but that dream was not meant to be…and now he is gone. It will be hard to go home without him, everything we see will bring back his memory, and with it pain and tears. We dread walking back into his empty room. We hold his Elmo close to our face in an attempt to capture his smell one last time. He will always be with us, and we look forward to holding him again in eternity. He was perfect and beautiful, and we could never of asked for more beautiful a child. We will wait until we can embrace him again, finally…together in heaven.

Monday, October 6, 2003 1:33 AM CDT

Entry 187—Oct 6,03/Post Transplant Day 228/Post Transplant Boost Day 166

Days have now turned to hours as Noah holds on by a very fragile thread. We are exhausted and drained; the stress is beginning to wear us down, the lack of sleep clouds our eyes and our stomachs turn at the thought of food. We stare off into space and often break down and cry. I can’t even talk to the doctors anymore without becoming a babbling idiot. This future is one we fought so hard to prevent, but it’s picking up speed now like a runaway train, and I fear we will lack the strength to prevent its devastating conclusion. We only want some time to see him off with our pastor, family, and Dr. Kurtzberg at his bedside. But I fear that even this simple request will be robbed from us too. The next few days will haunt us the rest of our lives; …peace is just too hard to be granted. The suffering will continue after the sufferer is gone. It appears… our last wishes will go unanswered; we will wait and see as we have done for the last year, always with the same results. We will probably be prevented from experiencing even a last kiss goodbye, a second of caring, a moment of love and peace with our precious son before he leaves. To me these things are not too much to ask for, as parents who have worked so hard… for so long. Unfortunately it looks like we will be stuck in the PICU, surrounded by four other families watching TV, talking and laughing, with doctors and nurses walking by the open bay, totally devoid of respect and dignity for our loss. I am sorry if this startles you, but I have to be true and honest on this journal; integrity is about all I have left.

Saturday, October 4, 2003 10:35 PM CDT

Entry 186—Oct 4, 03/Post Transplant Day 226/Post Transplant Boost Day 164

Today was a long day, spent in quiet rooms, with somber doctors pushing tissue boxes across tables that seemed to stretch for miles. Noah is still at 100% oxygen and it is beginning to burn and scar his lungs. By Monday, if they can’t bring the settings down, we are told he will become un-extubatable. We are devastated. Noah has been our sun, and we like planets, gravitate towards him. He has provided warmth in the cold, light to the darkness; our lives rise and set around him. All our mistakes, bad decisions, and the difficult times in life disappeared on the day he was born. Our wedding ring, round to signify eternity, became encrusted with a beautiful and timeless diamond; a perfect union became better than perfect. The trials and tribulations of his treatment were translucent, for we faced them together. We wonder how we will wake in the morning without the warmth of the sun on our faces; the nights already seem to stretch for eternity. Our sun is fading, and soon will become a beautiful star in the heavens, but the planets that have for so long enjoyed warmth and light will be left in cold darkness, to spin off into the void. We are almost blind; it is hard to see past our nose; we wander around the hospital in a daze, I need Debbie to help me remember where I parked the car…my thoughts scattered. We are on an island surrounded by sharks and the water runs red. Death is such a lonely place. We have been forced to realize that we need Noah more than he needs us. Life will never be the same; our souls our wounded and the scars will never leave us. We will miss our sun.

Friday, October 3, 2003 11:52 PM CDT

Entry 185—Oct 4, 03/Post Transplant Day 226/Post Transplant Boost Day 164

Noah has not improved; he spent the day on 100% oxygen and we were scared. They started to move to the oscillator, but by this evening he had picked his saturation levels up to the mid 90’s% so they held off. He can’t stay there much longer though, as that much oxygen becomes toxic after a while and will start causing other problems. Still no answers only more questions. Dr. Kurtzberg ordered high dose steroids today, so it looks like they are moving away from infection and trying something else. Noah has to respond soon, or we are going to run out of options. We need some guidance, some answers; we can’t make a decision now on an “I don’t know”. We cannot wonder the rest of our lives away if we failed our son. So we are watching and waiting. God has not directed us like he did the last time, but remains silent. Maybe he is watching and waiting also. He knows our resolve, but maybe there is a lesson that needs to be taught, maybe Noah’s work is not done yet. So we will wait and see what tomorrow brings.

Thursday, October 2, 2003 11:55 PM CDT

Entry 184—Oct 2, 03/Post Transplant Day 224/Post Transplant Boost Day 162

Today was a day of reflection. Noah’s support requirements have slowly increased, and we have watched the numbers and agonized with every digit raise. We transition from silence to discussion, and back to silence. Staring at the monitors and then glancing back to each other…our faces tell a year’s worth of story. We kiss his cheeks, rub his legs and arms, and run our fingers through the tussle of hair that has begun to grow back on his little head. We feel like we have to soak him in, enjoy every last second, savor his scent and softness. Leaving for doctors’ rounds is like leaving forever, and we agonize over it. Away from the hospital our thoughts remain behind, still at his bedside, our discussion always turns to him. We have had to do some hard thinking, and talk about feelings that have been silent for a long time. You have to prepare for the worst even though you hope for the best. There is no greater nightmare than having the doctors force you into a snap decision that you have not yet considered. We do not want to be shooting from the hip during life and death circumstances, because often you can’t take back a bad call. As of tonight we are still dealing with the unknown, and time is beginning to run short. A day of tests again produced a room full of doctors scratching their heads and walking out, leaving us frustrated and scared. We hope the next twelve hours will give us some answers. For now we are trying to enjoy Noah, and love him like there is no tomorrow.

Thursday, October 2, 2003 1:25 AM CDT

Entry 183—Oct 2, 03/Post Transplant Day 224/Post Transplant Boost Day 162

Often when I pray over Noah I don’t ask for much. What can I ask for that God does not already know? If we need a miracle, He already knows, and will provide, if it’s His will. Noah will be healed or we will lose him; that decision is out of our hands because… God is in control here. I can only ask that He makes His will known to us, time has become a tormenter. I know where to look for the answers I seek; God has always directed me to the right place in the Bible to deliver His message and I have always tried to listen. Now we ask only that His hand will move over Noah. I don’t know anymore were to go in the Bible, I cannot find the right prayer. I can only sit in the silence and let Him hear my heart…and it is breaking. How can you expect a parent to sit day after day and watch a beautiful innocent child suffer so much? We suffer too, and it goes on and on. We will not forsake our God but we long for His touch. Why does our beautiful jar of clay have so many cracks? We have held him close, padded and packed him, set him on a pedestal, would sacrifice our lives for him, and yet his agony continues. There is no more crying out to Him in the night, we are somber and quiet now, calm and attentive, waiting…for an answer. He has sent wonderful people into our lives to provide comfort; I have seen His touch through others. But now we wait, to see His hand glide over our son. We would give all we have to know His will, to see an end to our son’s misery. Tonight, as I kissed my son I told him it was okay if he had to go…but Lord why?

Tuesday, September 30, 2003 11:05 PM CDT

Entry 182—Sept 30, 03/Post Transplant Day 222/Post Transplant Boost Day 160

Noah has not made any real improvements. Since Saturday morning the PICU staff has attempted to wean him off of the ventilator, and sometimes he is able to wean down on some of the settings…giving us some hope that we are finally making a turnaround, but then he ends up leaping backwards and worsening again. His chest x-rays, although not completely clear, are not too bad at all, showing just a tiny bit of white in them, but he has had much worse and been outpatient breathing on room air or requiring little oxygen. However, he is having great trouble keeping himself oxygenated and has max’d himself out on his ventilator settings earlier this evening. In fact, we thought for a few minutes that today was the day, the day we would be losing him. He dropped his oxygen saturations down into the high 50’s% (normal 95-100%). They took him temporarily off of the ventilator and manually bagged him to try and increase his oxygen saturation level, and they could not get him any higher than the low to mid 70’s%. They placed him back onto the vent at high settings, closely max’d out on his pressures, volumes, and oxygen. He recovered for a while, but it definitely made our hearts jump and stomachs turn. He is a bit more stable, but continues to have trouble weaning. A skin biopsy was done of some bruise-like lesions that have popped up to check them as a source of infection. We won’t hear anything from this test for probably at least a day or two. He was running a high fever yesterday, last night and into today, but it finally broke. It is puzzling that whatever this bug is, that the antibiotics that he were on should have already begun to zap it and he should be improving, but if it is some sort of virus, then there is no treatment. We will have to just ride it out until they find something that could be the cause.
Thank you Eleasha, Carrie, Angelique (and mom), Howard, and Mackenzie for stopping in to see us these last couple of days. We appreciate the support and prayers. Happy Birthday (tomorrow) Kelly! We hope that you had a great trip!
We ask that you will continue to keep Noah in your prayers, and Tommy, Dylan, and all of the other kids that are in such great need of healing right now. God bless them for their suffering has been so great. We pray daily for mercy.

Monday, September 29, 2003 9:23 PM CDT

Entry 181—Sept 29, 03/Post Transplant Day 221/Post Transplant Boost Day 159

I have remained silent on Noah’s journal for quite some time now. I guess with all the turmoil and worry it has been hard to collect my thoughts. We seem to have become almost numb to the world around us. Every waking moment is spent focused on Noah, and lately during slumber my dreams are of him and his care. It has been a long and difficult year. Our lives have revolved around med schedules, labs, pumps, monitors, suction, and O2 requirements. When most parents would be worrying about whether or not they packed enough diapers or formula when they leave the house we are calculating if there is enough O2 in the tank to get him to clinic in the morning. Instead of trying to get our child to eat a particular baby food we have to calculate the flow time for continuous feeds on a pump. We are thankful for every moment we have with Noah but it is important to remember that it comes with a cost. I have been told that when we look back at this experience that we will remember the good times. I pondered that question for a while. The good times would have to be experienced by all of us, a time of happiness for the entire family. It can’t be something like Noah coming off of the ventilator, or having a medicine change, MRI or CT scan results… those are times of relief. So over the last year what good times do we have to remember? There is a time of about three minutes, three minutes in a year’s time when all of us were happy. Noah has smiled only four times in his life and once he laughed. We remember all of them; they are burned into our memory forever. Those are the good times we have to look back on, because it is impossible for us to look forward. Our future is unclear, we cannot plan for anything longer than a day’s worth of activities and even then it is unsure. You can get up in the morning at home with everything fine and by the evening you’re in the PICU watching them bag your son because he stopped breathing. This has been our life for a year now, and I often wonder how we have done it, and how much longer it will continue this way. We pray that someday we will get a break and finally find peace as a family once again, unplagued by sickness and tragedy. Hope has become a rare commodity and when it does come it is always followed by devastation. So, as Noah continues on intubation we will watch and wait… and see what the next twelve hours bring.

Sunday, September 28, 2003 0:46 AM CDT

Entry 180—Sept 28, 03/Post Transplant Day 220/Post Transplant Boost Day 158

Well, in our case, no news has not been good news. There has been so much that has taken place over the last couple of weeks. We were supposed to be discharged from the PBMTU on Tuesday, but they decided that after repeating the head CT scan, that Noah needed to be transferred to the PICU to have his shunt tapped and fluid checked. I think we already said this, but anyhow, after he stayed over night, he was released from there on Wednesday with the shunt results looking just fine. On Thursday we went into clinic, Noah still on 2L of oxygen and not looking the best. By that evening, he was retching some more, his oxygen saturations were dropping and his oxygen requirements increasing…up to 6-8L on facemask. We stopped his tube feedings that had been running and decided to page his PNP. After speaking with Dr. K, she called us back and said to take him off of the mask and turn down the oxygen to see what would happen. He did all right, although not as good as he has been in the past, and we left on his nasal cannula at 2L of oxygen. It appeared that stopping the feeds helped quite a bit, because he was no longer retching and was resting more comfortably despite turning down his oxygen. They told us to restart the feeds at 2 a.m. at half of his regular rate and see how he tolerated it. Well, we did not want to stay up until then, so we started them closer to 12:30 a.m. and within several minutes he was retching again and starting to desaturate. We immediately stopped the feeds and gave him some meds to help calm him, and some Tylenol since he had been crying and fussing some as if he might be in some pain. They had told us that if he persisted to have low oxygen saturations, to bring him into the hospital, but it seemed to do the trick and he rested for about 3-4 hours. By 4:30 a.m. though, he began his episodes again, with the monitor intermittently beeping from his low oxygen saturations. A few hours later it was a continuous steady beeping, as he remained low in the 80s% (normally at least 95-100%). We loaded him up and took him into clinic, where he was soon requiring A LOT more oxygen. Within a couple of hours the PICU fellow made a visit just to assess him, but it was decided that he should go to the PBMTU for the time being. We were admitted to the unit within the hour and throughout the day Noah had plenty of tests to try and determine the cause. His lungs sounded junky, but not as bad as they have at times in the past. His heart rate was bouncing around again though and they were thinking that maybe it was something wrong with his heart, seizures, or something wrong with his shunt. Neurosurgery was pretty convinced that the shunt was fine since it had just been checked not even 48 hours earlier. We weren’t convinced he was having seizures, and it was not really pursued. However, they did an ECHO and an EKG, both of which were normal. His abdomen and chest were x-rayed and his chest showed some small patches of ‘stuff’, but there were still no lab results to indicate exactly what was causing him to be doing this. By midnight though, he had worsened so much that he was taken over to the PICU and placed on a BiPAP mask, and later intubated. They suctioned SOOOO much yellow junk out of his lungs and the culture came back with a type of bacterial infection. FINALLY, an answer to what was causing all of his problems for the last few weeks. By this morning they had a clearer picture of which antibiotics to start him on that would actually be effective in killing off this bacteria. He is still on life support for now, but seems to be stable. It was a bit scary at first last night since he was needing 100% oxygen on the conventional ventilator and if he had worsened, he would have been heading towards the oscillator again. He is still very sick, but we are praying that he will make a quick recovery over the next couple of days. We are amazed that he was able to hold out for the last month though, what a tough little guy!
His donor cell test came back on Thursday afternoon, and it is down from 16% to 11%. Not much there, but I guess it is still doing enough right now and is better than 0. We are quite discouraged about it and were too upset to even write a journal about it the other day, but there is nothing that we can do. We have prayed and prayed and prayed and prayed some more, and they have not budged. In fact they seem to have dropped a little. There are no more words to express our frustration and disappointment that we have continually had to face this year, but we have no other option but to just put it out of our minds (if this is even possible) and look forward to tomorrow for better news. We simply do not understand. Contrary to this discouraging news, we did finally receive the MRI results from the MRI done about 6 weeks ago…finally! Noah has continued to remyelinate at an exceptional rate and is nearing the “normal” development curve for a child his age. This is truly AWESOME!!! Praise God for this glorious news! We are praying that there was little damage done to actual nerves while waiting for the myelin to regrow, so that he can regain abilities via physical, occupational, and speech therapy. If there is permanent nerve damage, then certain skills may be lost. Our only fear, the one that remains plastered in the backs of our minds, is that if he loses his donor cells then all of this incredible myelinating that has occurred will be in vain. If he loses his donor cells, then he loses the valuable enzyme that he needs, and his disease will once again begin to progress again. He is not eligible for another transplant, and this was our one shot at overcoming the death sentence that Krabbe had given him. Although there is always hope, we will forever remain terrified of this nightmare coming true. God has been merciful and gracious thus far, and we humbly ask that he will continue to heal Noah, including those stubborn donor cells. Thank you for the many, many prayers and words of encouragement. Thank you for signing Noah’s guest book and offering your support, we truly appreciate it more than you will ever know. God bless.

Wednesday, September 24, 2003 11:49 PM CDT

Entry 179—Sept 25, 03/Post Transplant Day 217/Post Transplant Boost Day 155

What a hectic and emotionally draining week! Noah was transferred to PICU yesterday evening, had his shunt tapped and spinal fluid samples taken to test for various labs. Everything went fine and the results came back looking good. No infection present, his fluid was crystal clear, and his protein level is now down to 126 (it was around 240 when he was diagnosed at 4 months old; normal is about 10-40). So even on his small amount of donor cells, he is still getting the job done. Go Noah!!!! And more importantly, Praise God!!! What a relief and an awesome blessing! The protein elevation is due to the myelin break down; protein is the byproduct of the broken down myelin, and it just collects and floats around in the spinal fluid. Occasionally, as in Noah’s case, it will clog up the aqueduct in the brain and require the need for a shunt to drain the fluid via another route. One way to know that the enzyme Noah needs is in his brain and working is by watching the protein level in the spinal fluid, and if it is dropping, then it is working in his favor to stop his disease progression. Whew!
Anyhow, that went well, and he was kept overnight. We took advantage of it and went back to the apartment to sleep. I don’t think either of us moved we were so dead tired. We arrived early to the PICU to wait to speak with the PBMTU team and they said that all looked good and we could take Noah home today. So we were discharged directly from the PICU, which is not something that is often done, and we took our sweet boy home. However, he needed more oxygen and sounded terrible AGAIN, so after a bit of convincing to the PICU docs, Noah received some albumin and a diuretic before we left. It definitely helped him out. He remains on quite a bit more oxygen than he has in the past, but we are hoping to get this lowered with some aggressive respiratory care and diligent suctioning over the next several days. He will remain on the antibiotics for about two more weeks, so hopefully this will kill off whatever bug got to him. We aren’t sure if this has knocked back our plans to go home next month (still no definitive date) or not, but we wouldn’t be surprised. We have not heard anything anyhow, so we are not holding our breath. Dr. K is still trying to arrange care for him back home and a transport flight as well. We shall see what happens with all of this.
As for his MRI, still no results…not that it matters. We see improvements in him that tell us that he must be still remyelinating more, but it would of course be nice to see on paper. His donor cell test results we hope will be in by Friday. We continue to pray!!!! Again, we want to say THANK YOU for all of the birthday wishes, cards and gifts that were given to Noah in celebration of his first birthday! We are so touched by the outpouring of love and support that we receive each day. God bless you for your kindness.
We want to wish a HUGE congrats to Ashleigh who is now outpatient. As well, we want to continue to ask for your prayers for Dylan who is having some complications and has not yet been able to start his chemo, and for the Trimper family whose two youngest children, Maddie and Sam, who were admitted last evening to start the transplant process. We wish them all the best and continue to keep them in our thoughts and prayers.

Tuesday, September 23, 2003 3:04 PM CDT

Entry 178—Sept 23, 03/Post Transplant Day 215/Post Transplant Boost Day 153

Well, our supposed discharge day has turned into a mess. Still concerned about Noah’s heart rate lability, the doctor on the PBMTU decided to send Noah for a head CT scan to assess his shunt and CSF in his brain. As well he had an EKG to check his heart. Noah has been fussier today and has been retching some as well. When we were all packed and ready to receive the words, “All right, you can go now, see you in clinic tomorrow for follow-up”, and we were told instead that Noah was being moved back to the PICU and neurosurgery would be there to tap into his shunt, assess the pressure in his brain, retrieve samples of spinal fluid for lab cultures. Our worst fears are that his shunt has failed, and/or he has an infection. If either one of these is true, then Noah will not be going home any time soon. Please keep praying for him. Will it ever stop??? We cannot take anymore. We just can’t. We will update when we can, but please just pray.

Tuesday, September 23, 2003 1:49 AM CDT

Entry 177—Sept 23, 03/Post Transplant Day 215/Post Transplant Boost Day 153

We have not had much time to update, but Noah was in the PICU overnight, and was transferred over to the PBMTU on Saturday afternoon. He was taken off of the Dopamine drip when we returned to the bone marrow unit and over time, his blood pressures are now creeping back up into the “too high” range, and he now is having to be put back onto his anti-hypertensive medications for high blood pressure. We were hoping that he would not need to be started on them again, but it looks as if he will. He is already back on one of them, and they may be starting him back on the second one by later this morning. His heart rate is no longer sustained as high as it was, but is jumping around now and doing some crazy things like dropping as low as in the 50s and as high as in the 160s. He has developed a rash now, but it is hard to decipher exactly what it is that is causing it. It could be a number of things, such as reaction to a medication being the most likely, donor cell growth the least likely. We drew his donor cell test this morning and it will be sent off. We may receive the results as early as this weekend, but it may take until next week to hear the news. We are praying that the results show he is growing more donor cells. He is having some respiratory issues…as we all know Noah does not do well when he has too much fluid on board. Well, when his blood pressures were dropping so low on Friday, he was given a total of 4 fluid boluses and a unit of red blood cells and platelets. By early Sunday morning it had hit his lungs, and he needed a lot more oxygen and he sounded like he was drowning again. We were having to basically stand at his crib side all night and day with the suction in hand since he was requiring it so frequently to rid him of the mucous/fluid. His weight had increased by almost an entire kilogram (about 2 pounds of extra fluid). He was started back on his diuretics again and they are starting to help him some. The tentative plan is to discharge him later today, but with his heart rate and blood pressures acting up, and this unexplained rash that he has developed, who knows what they will decide upon. So much for starting off his second year of life in a good way…not even a week into it and he ended up in the hospital. None of his cultures have grown anything, so there is no actual infection declared as the cause of this last episode that occurred on Friday night. The only thing they have not done is draw spinal fluid from his shunt to test it for possible bacteria. We may bring this up tomorrow, although we already did earlier, but they weren’t so sure that he needed to have this done. Neurosurgery would need to be called in to do this procedure. Thank you all for your continued support and prayers. Noah really needs them right now. We ask that you will continue to remember the other kids and their families too, there are so very, very many in need of prayers. We wanted to mention a huge congratulations to Ashleigh, who will be discharged from the PBMTU tomorrow!!!!! Yea Ashleigh!! We will continue to be praying for her as she begins her outpatient clinic phase of the transplant process.

www.caringbridge.org/nj/ashleigh.ryann

Dylan really needs some extra prayers right now! Please remember him in your prayers too. Thank you.

www.caringbridge.org/europe/dylan

Saturday, September 20, 2003 8:00 AM CDT

Entry 176—Sept 20, 03/Post Transplant Day 212/Post Transplant Boost Day 150

Noah’s in the PICU, went in last night, due to sepsis…blood pressures 60-50s/20-30s. Heartrate 180s-200s. He was put on a Dopamine drip to help raise his blood pressure, and they are currently weaning him down. He got blood and fluids to help raise his blood pressure and it seems to be helping. We hope to have him transferred to the PBMTU today, and then maybe released tomorrow or Monday. He is on some major antibiotics and they seem to be doing the trick, his labs are showing that they are. Would appreciate your continued prayers. Thank you. We will update as we can. God bless.

Thursday, September 18, 2003 9:24 PM CDT

Entry 175—Sept 18, 03/Post Transplant Day 210/Post Transplant Boost Day 148

No worries, we are all safe and dry. Although we weren’t that close to the coast, about 2 hours drive away, I think, it really wasn’t that bad at all. I guess with the trade winds always blowing in Hawaii, sometimes up to 30mph or higher, and the winter’s high surf at North Shore (sometimes swells up to 30 feet high—a surfer’s dream), it wasn’t as impressive as we had expected…which is a good thing! We had heard that there were no deaths or injuries reported yet, which is the most important thing. We will pray that there aren't. There were a lot of leaves and branches around when I drove to the grocery store…yes, I did, but it wasn’t bad, just a little windy and rainy. Anyhow, we are fine!
Noah has been doing about the same the last couple of days, still needing that darn oxygen though. That is our goal for this week, to get him off of that and back on room air. Tuesday is our BIG lab draw day, we will draw his donor cell test!!! We are a bit on edge, hoping that his donor cell count has increased!!! Thank you for your continued prayers and support!
We want to ask that you would remember a few others in prayer. Noah’s Krabbe buddy Ryan, was released from the PICU in his hometown after having pneumonia. We ask that you will pray for his recovery. Ashleigh will be discharged from the hospital this next week…yes, after just getting out of PICU last week!!!! WOW!!! Please continue to pray for them as they make this HUGE step forward in her transplant journey. Please remember Dylan, who is going to be starting chemo soon, and has already had an extremely rough time. Please remember Manny and his family in your prayers, as they are having an extra hard time right now, besides having to care for Manny’s needs. There are so many kids that need a lot of prayer, and we have not mentioned them by name, but please keep them in your prayers. God bless.

www.caringbridge.org/sc/mannymiramontes

www.caringbridge.org/nj/ashleigh.ryann

www.caringbridge.org/europe/dylan

Wednesday, September 17, 2003 11:29 PM CDT

Entry 174—Sept 17, 03/Post Transplant Day 209/Post Transplant Boost Day 147

“There’s a hurricane a-comin’!” We have never been in a hurricane, but we have had a lot of “first’s” this year, so why not? We don’t have to draw labs or go to clinic until Friday, after the storm has passed, so we won’t have to get out in it. I think that everyone at the clinic worked hard to make sure that no one had to get out in the storm if possible.
As for Noah’s big birthday, well Noah had a rough day on Monday, running fevers throughout the day and into the night. We paged Dr. K to see what she wanted us to do, so we were told to wait it out until the morning if possible, since Noah was already on one antibiotic. Noah had a rough night, with constant crying until about 4:30 in the morning…it was a bad reminder of how he used to be every day, all day, before his transplant. He used to cry about 20 hours a day, inconsolably, what a nightmare! We arrived early at clinic Tuesday, after a very long night. He had another chest x-ray done, blood cultures, and his other labs done. He received 5 infusions, a lot more than usual, and we had about a 9 hour clinic day. What a birthday, huh? He is still on oxygen, not sure what it is that is keeping him on it, his x-ray was relatively clear, and he doesn’t sound too bad. We started him on a new antibiotic to see if this will help him. There were no medication changes other than that one, and no results on his MRI yet either. Next Tuesday morning I will draw his blood work for his donor cell test! We are praying that the results are good. Noah has not made too many more steps forward, but he continues to cough and move his limbs occasionally. His eyes will crack open occasionally, more often than they used to, which is good to see. Still no word on the exact time of going home, but we are not too worried about it right now. We appreciate all of your amazing prayers, support, encouragement and birthday wishes. We were so astounded by the staggering numbers of those of you who wrote your birthday wishes in his guestbook! Thank you so much! We cannot begin to express our gratitude for your thoughtfulness. We appreciate it so much. Although Noah cannot read, we are able to share with him how much support he has in his favor. God bless you. We kindly ask that you will continue to remember his needs in your prayers. Thank you.

Tuesday, September 16, 2003 6:34 PM CDT

Entry 173—Sept 16, 03/Post Transplant Day 208/Post Transplant Boost Day 146

HAPPY FIRST BIRTHDAY TO YOU, HAPPY FIRST BIRTHDAY TO YOU! HAPPY FIRST BIRTHDAY DEAR NOAH, HAPPY FIRST BIRTHDAY TO YOU!!!!!!! YEA NOAH, YOU DID IT!!!!!!

HAU’OLI LA HANAU!!!!!

Here is a birthday story just for the occasion!

Oh, the Places You’ll Pop Up!!
By Dr. Seuss

CONGRATULATIONS!! Today is your day. You’re off to Great Places! You’re off and away!

You have brains in your head. You have feet in your shoes. You can steer yourself any direction you choose.

You’ll look up and down the streets. Look’em over with care. About some you will say, “I don’t choose to go there”.

OH! THE PLACES YOU’LL GO!!! You’ll be on your way up! You’ll be seeing great sights! You’ll join the high fliers who soar to high heights.

Except when you don’t. Because, sometimes, you won’t. I’m sorry to say so, but sadly, it’s true; that Bang-ups and Hang-ups can happen to you.

But on you will hike. And I know you’ll hike far, and face up to your problems whatever they are.

Just be sure when you step. Step with care and great tact, and remember that Life’s a Great Balancing Act.

And will you succeed? YES!! You will, indeed!! (98 and ¾ percent guaranteed.) KID, YOU’LL MOVE MOUNTAINS!!!!

You’re off to Great Places! Today is YOUR day! Your mountain is waiting. So Noah, …get on your way!!!

What a HUGE milestone today is for such a small boy, whose life has only begun. His future is so uncertain, and we cannot be sure of what will happen or how things will turn out, but we thank God for each day that we have to hold him and tell him how much he is loved. There are so many memories that I have over this last year…most of which break my heart. Our son’s baby book is filled with photos of him in the hospital, with tubes, wires, drains, monitors, machines, scars, dressings and bandages. Our hearts break at the many milestones mentioned in the book that are left empty. You see, Noah never learned to roll over, grab at toys, babble and coo, sit up on his own, crawl or pull himself to a standing position. He never grew in his first tooth. His doctor visit page exceeds the norm and some of his milestones include his first day of chemo, his transplant, and his first show of white cell growth. The goal that most parents have for their one-year-old is to see him up and walking soon, our goal is to go more than a week without needing platelets or blood. Looking beyond all of this we know that Noah would not be here if he had not gone through all of the pain and suffering that he endured this last year, and we are so blessed to still have him with us to celebrate this special day. Noah has such a long journey, with a road hazed with uncertainty of where it will lead him. Our only peace is faith that God will heal his sweet little body from all that has been stolen from him, in hopes that his future years will be so much brighter for him. We hope to one day be able to fill in those milestone lines in his baby book…that Noah may one day be able to use his hands, arms, feet and legs. That he may be able to one day eat from his mouth and not from a tube. That he may be able to see again. That he will be able to begin to smile, laugh, coo and babble. We have hope for his future and pray for God’s grace. He has taught so many people here at Duke so much. If you can take anything from his life, please remember that life is a special gift, not to be taken for granted… it is full of uncertainties and at any moment your life or someone you love, could be changed forever in a way that you could never imagine as ours have this last year. Wake up each day, and live it as if it were your very last day on this earth…you never know, it may very well be. With Noah’s donor cells still at such a very low percentage, we have had to do so. We continue to live each day, knowing that those small amount of cells, yes simple cells, are what is keeping him alive with us today. We know that he has lost them before and it could happen again…so each day that we have with Noah is a day spent as if it were his last. It is exhausting, but oh how much more we appreciate life now, and realize how many ridiculous things we used to focus on. Noah has blessed our lives beyond measure and taught us about love, faith, hope, pain, fear, and the value of family and friends. There are some that have given us so much support continuously throughout this entire journey, some more than others, but we appreciate all of the encouragement that we have received to help us get through this difficult year. We will be heading home ‘soon’ and starting another chapter in our lives. We continue to pray that this chapter includes Noah’s healing and improvement. We thank you for your continued prayers and support, and THANK YOU for the special birthday prayers for Noah today!!!! God bless.

Sunday, September 14, 2003 12:59 AM CDT

Entry 172—Sept 14, 03/Post Transplant Day 206/Post Transplant Boost Day 144

Let us start by saying a HUGE “THANK YOU” to Kelly for hosting Noah’s birthday bash this weekend! It was a great success and we are SO VERY thankful for your kindness and generosity. We had a wonderful time! Thank you so much to all of those who could make it and for the birthday gifts that you gave to Noah! You certainly did not have to or need to do that, but we are very grateful for your generosity. Noah had to get blood yesterday…and we thought we were not going to make it to the party, or at least an hour or two late! But, the PNP had the nurse give Noah a partial unit to carry him through and it did not require the full 3 hours to infuse. His hemoglobin had dropped to 7.9! This is pretty low for him, not as low as it has been in the past though…he dropped to 4 the night he was admitted to PICU (too scary to think about, this was dangerously low). Anyhow, he has been recharged for the time being. We had a great time socializing with everyone. Thank you to Elijah, Degan, Brent, Amanda, Mackenzie, Sam, Max, Maddie, and Daryn for coming to Noah’s party! We were sorry that Dylan and Ashleigh could not make it too, but they are still inpatient right now. By the way, Ashleigh is now back on the PBMTU, with a WBC of around 2.0…and doing great! Wow! Thanks for all of the prayers for this little princess, and praise God who answered them! Please continue to remember her in prayer, and please say some extra prayers for Dylan, who has had some big setbacks and has not yet started chemo yet. (See web pages below).
We are just a couple of days away from Noah’s actual first birthday and BIG DAY of PRAYER!!! Keep spreading the word about it…we hope to raise the roof of Heaven on this day!!!!! Noah has begun to make some small improvements…as I have already mentioned in the last couple of journals. He is starting to cough occasionally and gag, well last night he kept his dad up most of the night because of his coughing!!!!! How funny and outstanding is that!???!!!!! Go Noah Go!!!!! We would rather lose sleep because of Noah keeping us awake coughing, than lose sleep from his monitors going off and him not being able to cough. His eyes are mostly closed still, but he has been opening them briefly more often now. We are desperately praying that his donor cells are growing like weeds inside him, and that his little body continues to heal. We praise God for these small miracles and humbly ask that He will continue to heal our sweet baby. Thank you for all of the prayers and encouragement! We are so very grateful! God bless.

www.caringbridge.org/europe/dylan

www.caringbridge.org/nj/ashleigh.ryann

Thursday, September 11, 2003 8:50 PM CDT

Entry 171—Sept 11, 03/Post Transplant Day 203/Post Transplant Boost Day 141

Noah’s labs were pretty good this morning and so we did not have to go to clinic. Yea Noah! He has been pretty much the same the last few days, without any bad surprises, and actually has given us a few good ones with his occasional cough/gag and moving his legs a small amount. His EEG showed no changes from the ones he had done while he was inpatient, …which is of no real surprise, but we are pleased that it showed no seizure activity! Kelly has graciously offered to host Noah a first birthday bash this Saturday, so again all of the Krabbe families are getting together to celebrate the occasion. We are excited about this celebration and plan to take plenty of pictures to post. His actual birthday on next Tuesday is our big prayer day. I only bring it up again in case there are any new readers who may not be aware of our ‘prayer plan’. We will likely have another small celebration on his actual birthday as well, but we will be in clinic that day. Thank you for all of the prayers and support, and for spreading the word about Tuesday (9/16). Noah has been making small tiny improvements and we are excited for what we will see unfold in the near future in regards to his healing. We are desperately praying for His healing touch! Praise God for the small improvements He has allowed Noah to make already. Please continue to spread the word, and to pray for all of the other kids that are in need of healing as well. There are so many kids that are so very sick. We also want to make a very special prayer request for Ana and Luis, who lost their daughter this morning to Krabbe. Elly suffered for many months from this horrible disease and today she flies free from it and all its pain. Fly Angel Elly, fly sweetheart. May God show His mercy. We pray on this special anniversary of a tragic event, for those families who lost members in the attacks on our country. We will never forget. God bless.

Tuesday, September 9, 2003 11:55 PM CDT

Entry 170—Sept 9, 03/Post Transplant Day 201/ Post Transplant Boost Day 139

Wow, over 200 days already! Amazing how time has gone by, and looking back there has been so much that has had happened, unfortunately most of which we would like to forget. However, we are looking forward to a brighter future for Noah, and praying desperately for God’s blessings of healing. We must give some praise to Him, as Noah has been a bit more awake the last few days…giving us more coughs today than he has in the last month I think, starting to have a bit more pronounced gag, and moving his legs a bit now (ever so slightly). These are very tiny improvements, we are not talking big, but to him this is a HUGE deal! Praise God for this! Isn’t it amazing the little things taken for granted? Well, we have had to learn not to on a daily, hourly, basis. Noah will not have his donor cell test done next Tuesday, on his birthday, but rather on the Tuesday to follow (9/23)…so with all of the prayers, this will allow God another week to help them grow. Please continue to spread the word on Noah’s BIG prayer day, his first birthday…next Tuesday, 9/16!!! Thank you to all of you who have already done this! What an amazing day it will be! No other changes made today on any medications or treatments. We did receive the results back from his nerve conduction study…and his results are worse than the one done in January…but that was 9 months ago, so it is really not much of a shock considering how much the disease has progressed in him. When we arrived Noah was eating from a bottle, smiling, starting to laugh a little, moving his arms, kicking his legs, holding his head up well, maintaining great eye contact and tracking objects well. He was showing symptoms…extremely irritable, posturing almost all of the time if not medicated, cortical thumbs, but he seemed more like a normal baby to a certain extent. Although we are counting our blessings that he is here with us today and about to celebrate his first birthday, we mourn the loss of the little boy that we knew…and all that he has lost going through so much pain to get to this point. So we continue to pray for God’s healing.
He also had an EEG done today, again part of his six-month post transplant studies, and we have no word on that yet. No word on the MRI…I guess the specialist that reads this particular type of MRI is one of a kind and is in no hurry. So we continue to wait. Maybe it will be back next week and will bring us some good news on Noah’s big day. Overall, Noah has been doing better, not needing any oxygen today, but is back on it tonight. He is still having pretty bad withdrawals from the methadone, so when he gets worked up, he needs a small amount of oxygen.
Thank you again for all of the support and prayers. Thank you Kelly for all of the hard work that you do taking care of all of us Krabbe families that come to Duke. We appreciate you so much! Thank you to the Trimper family for the “Kick MLD in the butt” T-shirts!!! We love them and will wear them proudly!!! There are some other bone marrow kid birthdays this month that we want to mention, Brent who just became a teenager, and Tommy, who will be having his third transplant on his birthday…please keep him in your prayers that this one does the trick! He and his family have been through so much. If we have missed anyone, Happy Birthday!!! God bless.

Sunday, September 7, 2003 10:43 AM CDT

Entry 169—Sept 7, 03/Post Transplant Day 199/ Post Transplant Boost Day 137

We have not made a journal entry in a few days, but nothing has been going on. Noah, still on his small amount of oxygen, remains relatively the same. We went to the Ronald McDonald House here at Duke where some of the other Krabbe families are staying while their kids are inpatient. We had a big Krabbe party, with eight Krabbe families there. It was so nice to be able to all get together. As I sat there, I just took it all in. It was weird how a couple suction machines going during dinner didn’t seem to faze anyone…it was as if it was commonplace, where at other places in public it might be considered rude or inappropriate. It is amazing the bond that we have. Dr. K mentioned that her Krabbe families are the closest-knit families of any she has seen. Some of this is facilitated through Hunter’s Hope and the important role they play in all of our [Krabbe] lives, but most is because of our children. We are blessed for having these close friends that truly relate to what we are going through in some sense.
Thank you for all of the prayers and support. Just a little over a week before Noah’s big day. We are praying that his donor cells are growing wild within him and that the new donor cell test that we hope to do next week will be much improved. We keep hoping and praying.

PLEASE PRAY: Ashleigh ran into some respiratory trouble from her mucocitis and has been placed in the PICU this morning. She is stable and I made it by there this afternoon to check on her and her mom, but they do need a lot of prayer right now. Please pray that Ashleigh's mucocitis heals and she can be extubated soon and return to the PBMTU. God bless them.

www.caringbridge.org/nj/ashleigh.ryann

Thursday, September 4, 2003 4:01 PM CDT

Entry 168—Sept 4, 03/Post Transplant Day 196/ Post Transplant Day 134

Noah’s Day of Prayers for Hope and Healing on Tuesday, 9/16/03. Noah will turn 1, tell everyone you know and ask them to please pray!

JUST ONE MORE THING...DUKE TELETHON GOING ON RIGHT NOW TO RAISE FUNDS FOR KIDS THAT COME FOR TRANSPLANT...SUCH AS FUNDING RESEARCH FOR STEM CELL TRANSPLANTS, FOR KIDS JUST LIKE NOAH.

The Duke Children's Hospital & Health Center is having a telethon to raise funds. We are asking those that are financially capable, to give even a small donation, as their hearts are led to do so, and to please call the telethon number to offer your pledge to help out the kids and families that come to Duke for transplant and care. Thank you for your support!
Toll free telethon number good until Sunday (9/7/03) evening: 1-866-684-1015

Noah needed platelets yesterday, so went in for a brief clinic visit to charge him back up for a week. He is still on that ½ L of oxygen, staying relatively the same, but at least not getting any worse. We are hoping that he will finally be able to kick this bug that has got him feeling so yucky and no longer need the oxygen. He is having some withdrawals from his methadone, but we are almost totally weaned off of it, just a couple of weeks more and he should be finally off of that stuff. That will be one less med we are putting into his little body. Our ultimate plan is to go ‘el naturale’…no meds! Along with his donor cells, this is a huge task, but we are hopeful we can get there eventually. As for his donor cells, this is one special prayer request that we have, that God would grace Noah with 100ngraftment of pure donor cells. As well, we are asking that Noah’s body will heal from the all of the trauma that it has been through this last year, such as his eyesight would return, he would regain the ability to effectively clear his own airway by coughing, gagging, and swallowing, he would be strong enough to move his arms and legs again, and that we would once again be able to see him smile. As of now, Noah is severely affected by this terrible disease, these words from Dr. K herself on Tuesday’s clinic day. We did not expect him to come out of this transplant like this, and we don’t think she did either since he was doing so much still before transplant. He is devastated, and so are we, but he is our only child, our son and we love him with every ounce of our beings and cannot imagine not having him in our lives no matter his abilities or lack there of. We know that there is power in prayer and God performs miracles…so we are desperately praying for Noah to be blessed with a miracle. That is why we are asking for your help on his first birthday. We are hoping that Dr. K will draw another blood sample on that very day to assess his donor cell status again since having changed his immunosuppressant dose. We want to say thank you, and it hardly seems enough, but it is truly heartfelt and we really do appreciate all of the support, encouragement and prayers that have been offered us. We are so very, very grateful to those of you who have graciously and generously given monetary gifts through fundraising to help relieve some of the financial burden for us…THANK YOU so much…it has helped us out so much! God bless you.

We want to wish Princess Madi the very best as she has now gone home home!! We will keep her in our prayers. We want to mention a prayer request for Cody, as he underwent surgery this week. We also want to mention Tommy, who has begun chemotherapy and will undergo his THIRD transplant in a couple weeks. Please keep these special kids and all of the others that are in need as well, in prayer. Thank you.

www.caringbridge.org/nc/princessmadison

www.forcody.org

www.caringbridge.org/ca/bennettboys

Nolan needs some extra prayers too, for he may be having a bout with GvH right now.

www.caringbridge.org/co/nolanramsey

Also, a young boy named Conor and his family could use some extra prayers right now too.

www.caringbridge.org/canada/conorford

Tuesday, September 2, 2003 8:37 PM CDT

Entry 167—Sept 2, 03/Post Transplant Day 194/ Post Transplant Boost Day 132

Well our day at clinic with Dr. K was one of our fastest ones ever! We arrived at noon and were out in three hours!!! Dr. K lowered Noah’s methadone and steroids finally after a few weeks of staying put at where he was. No other changes were made. Still no word on the MRI results…we are waiting with bated breath to hear the news. Noah is still on a small amount of oxygen to maintain his oxygen saturations, but seems to be improving slowly from last week’s episode. Dr. K believes that he was getting septic, having some sort of lung infection, but we were able to catch it early enough to keep him from getting any worse and having to be admitted to the unit. Thank God for that!!! We are so blessed that Noah did not get any sicker than he was. However, there was no word on when the next donor cell test will be done, but we are hoping in a few weeks. She did say that she is still working on the arrangements to get us home.
As you probably know from the previous journal, Tuesday, September 16, is Noah’s first birthday, …a huge milestone for him! We are asking that on his special day, you would say a special prayer on his behalf, for God’s blessings of healing on his little body and for his donor cells to grow. We are asking that everyone mention our special prayer request to your friends, family members, fellow prayer warriors, co-workers, acquaintances, etc. We are hoping that our coming together to say some extra prayers on this day will make a big difference for Noah as he begins his second year of life… prayers for God’s blessings of healing, renewal and life from here on out. Please mark your calendars for this very special day…Noah’s Day of Prayers for Hope and Healing (9/16/03). Thank you for your continued support and encouragement. God bless.

Sunday, August 31, 2003 9:26 PM CDT

Entry 166—Sep 01, 03/Post Transplant Day 193/ Post Transplant Boost Day 131

Noah will turn one in a couple of weeks. In many cultures the passing of a child’s first birthday is cause for great celebration. This is particularly true in the Hawaiian culture. The entire family and friends gather at the beach for music, food, and festivity. This celebration can go on for days, with pig roasting, dance and song. This tradition came about because before the advent of modern medicine, many children did not make it to their first birthday. Is it the passing of time that they celebrate or the relief that the time of worry has passed? I would like to think the celebration is for the future. Looking forward to life, and all the possibilities it will offer their child and family. On Noah’s birthday we will be in clinic, far away from the blue skies and clear water of Hawaii. There will be no great gathering of family, no music, and dinner will probably be picked up at the drive-thru on the way home. We will celebrate though, not the passing of a year but the hope the next will bring. Noah’s first year of life is a blur to us, it runs together and haunts our dreams, and so we will be glad to put it behind us. We are thankful for every day with him and are hopeful for what the future will bring. So on Noah’s birthday we will hold him tight and put the past behind us, continually looking forward to a brighter day.

Friday, August 29, 2003 4:36 PM CDT

Entry 165—Aug 29, 03/Post Transplant Day 190/ Post Transplant Boost Day 128

No results on the MRI yet, but we did receive word on his donor cell test that was taken earlier this week. This test shows that his donor cells are at 16%, so considering the margin of error, he basically has been stagnant between 13-19% since early June. Now at first I was very upset…but I know it could be worse. I suppose it is the old principle, “…glass is half-empty, glass is half-full”, and I used to be more optimistic, but this morning I was not in the mood to entertain optimism. However, as the day passed by I was able to be a bit more objective and realize that we really have not gone up or down in a while, so although we have not gained any ground, we have not lost any either. So our plan is to increase his immunosuppressant and try it again in a few weeks to see where they go, which hopefully will be up, up and up. He is still on oxygen, but seems to be slowly improving. We are giving him IV antibiotics here at home now and we should hopefully not have to go to clinic tomorrow, which will be nice after 11 days straight. We are so very grateful for all of the prayers and support that you have continued to provide us. God bless.

Thursday, August 28, 2003 7:59 PM CDT

Entry 164—Aug 28, 03/Post Transplant Day 189/ Post Transplant Boost Day 127

Noah had another rough night, with his oxygen requirements increasing as the hours passed by, ending up on 2L by morning to keep his saturations above 92%. We went to clinic for our antibiotic infusions and were given the okay to go home, so it was only a three-hour visit. However, Dr. K said that she thinks that Noah was probably septic (infection in his blood) and had a respiratory infection of some sort since his blood pressures were running so very low, his need for oxygen increased and he has been lethargic. We are not so sure since his blood cultures have been negative thus far, but who knows…anything is possible. He seems to be improving slowly and we will return to clinic tomorrow for more meds. We are hoping to have his donor cell test and MRI results tomorrow, or at least one of the two. We are praying that we receive better news this time around. We appreciate all of the prayers and support. We want to offer a special “Thank You” to the congregation of First Church of the Nazarene in Mexico, MO for the special donation. We cannot express enough gratitude for your generosity and kindness. God bless you.
We ask that you will continue to pray for Noah and all the kids in need of prayer who remain very sick. Thank you.

Wednesday, August 27, 2003 8:13 PM CDT

Entry 163—Aug 27, 03/Post Transplant Day 188/ Post Transplant Boost Day 126

Well, we were planning on trying to stay positive, until our night became very ‘hairy’. Noah’s monitor kept alarming and the ½ L of oxygen he was on became to be not enough to keep him above 90%. However, the gauge on our big tank only goes up to ½ L. We have some small portable tanks that go up to 1L, but only last a few hours. However, given his need we changed him to a small one and prepared for another long day in clinic. As we packed up his bags, meds, suction, nebulizer, etc., we silently kept our worst fears to ourselves, but both thinking for certain that Noah would be admitted and he was headed down a bad road. Before leaving, his oxygen saturations were around 89-91% on 1L of oxygen, so it seemed he was getting a bit worse. When we reached clinic, fortunately our PNP had already briefed everyone and we were weighed in immediately, vitals assessed, and put into a room in the RDH, where Noah was hooked up to the monitors. His blood pressure was 63/32…Not a good sign!!!! He looked in distress, with retractions, he was lethargic, and showing other classic signs of distress. His labs returned with his white cell count at 1.3 after being at 5.8 yesterday. THis too was quite alarming. They were really concerned about the drastic drop and repeated this lab later in the day, and the results came back as 3.1, so maybe the tech who entered the results into the computer switched the numbers and entered it wrong. By the time he was in the room his requirements had increased to 2 L of oxygen to keep his saturations around 94%. The only thing that came to mind was “Here we go again!”. He received a couple of antibiotic infusions, platelets for his count of 14, and another med infusion. We sat in limbo all day, unsure if he was going to be admitted to the unit or not. We didn’t find out until around 6:00 pm that they were going to let us go home and just wait to see how our night went since they felt that he was doing a bit better. Fortunately, the company that supplies Noah’s respiratory care needs came out to give us a new gauge. He is now on 1L of oxygen and no longer in distress. His blood pressure before leaving clinic this evening was about 88/45, which is much better. He is still pretty lethargic, and we won’t know if he is positive for any infections until his blood culture and respiratory viral battery culture results return in a few days, giving them a chance to grow something. His blood gas was all right and most of his other labs were good as well. His chest x-ray has shown pretty much what it has shown for a long time, a small amount of atelectasis in his right upper lung, which is nothing really significant for him. We will be returning to clinic everyday for at least a week for antibiotic infusions. We are praying that this is nothing, that he will clear up from whatever has caused this and we will all be able to have some days of good rest. Thank you for your continued support and prayers. God bless.

Tuesday, August 26, 2003 10:10 PM CDT

Entry 162—Aug 26, 03/Post Transplant Day 187/ Post Transplant Boost Day 125

Another long day in clinic, but it went fine. At first, this morning Noah sounded pretty bad and by the time we arrived in the Rainbow Day Hospital he needed oxygen. He received a couple of infusions and then he was given a huge dose of a diuretic, much higher than his normal dose. His weight was back up quite a bit, but we are hoping that by tonight he will be a bit better. Dr. K says to just put him on some oxygen at night to keep him comfortable, to let us all sleep, and that it certainly won’t hurt him. We are attempting to think positive this week about the tests results that we are anxiously waiting for, which are his donor cell test that was repeated and his MRI diffusion study. We hope to get both of these by the end of this week and are trying to put aside any “stinkin’ thinkin’”. I suppose there is no reason to be down in the mouth until we actually receive the bad news and we have not yet received any this week, really. And of course we are praying for good news! His ECHO is tomorrow and we will check his labs tomorrow, because he may need platelets. It seems that since changing his CMV med, he is growing his white cells a bit better and it also may allow him to hold on to his platelets longer.

Dr. K is trying to work out the issue of our return, checking into setting up care with the Hem/Onc team at Kapi’olani, where I work. That is fine with us as long as we can get back home and Noah is taken care of. She will be in close contact with them, sort of guiding his care from afar since they don’t even know about his disease, but we are absolutely delighted to jump at this opportunity to educate them. We know that when she sets out to do something, she always seems to get it done. She is definitely an asset to have working in our favor. Thank you for all of the wonderful support and prayers. Thanks to Matthew’s family for the chocolate…purely soul food! We want to wish our Krabbe friend Daryn and his mom Theresa a huge congratulations on their escape from the PBMTU, as they were discharged yesterday! Yea Daryn!!! Hope to see you in clinic soon! We also wanted to send out a few special prayer requests for Dylan & Ashleigh, our other Krabbe friends starting the transplant process. We want to mention Tommy B., princess Madi, and Manny too, who we are aware could also use some extra prayers. There are so many kids that need a lot of prayer and I know that we have only mentioned a few, but we are definitely praying for them all. We want to ask that you would remember a fellow Krabbe family who is grieving the loss of their daughter Hayley, who passed away the end of this passed week. God bless them, God bless these kids & families, and God bless you.

Monday, August 25, 2003 10:49 PM CDT

Entry 161—Aug 25, 03/Post Transplant Day 186/ Post Transplant Boost Day 124

Tomorrow is our big clinic day. Today we were able to stay home, but for a few hours we thought maybe we would be going in to clinic, because Noah was having a problem with his mucous and fluid shifting again. Same old stuff. We gave him some extra doses of diuretics and we are hoping that by later tonight they will kick in, he will lose some excess fluid and we will all have a pleasant night. He will probably have his donor cell test redrawn tomorrow since last week’s may have been an error. He is also supposed to have some immune studies done, but they probably won’t do both since it will require to take SO much blood from him all at one time. The only other test scheduled for this week is his ECHO to check his heart function. There is no concern about this, but it is just part of the post-transplant check-ups that are done. Thank you again for all of the tremendous support and prayers during the last few days. We have really needed it. We are crossing our fingers and praying hard for better days ahead. Thank you Maggie for all of your help this weekend. God bless.

Sunday, August 24, 2003 10:38 PM CDT

Entry 160—Aug 24, 03/Post Transplant Day 185/ Post Transplant Boost Day 123

Well we went to clinic again today for Noah to get some blood, fearing that the large infusion would again get him into trouble, but we will have to wait and see how the night goes. They gave him an extra diuretic so we are crossing our fingers. What a weekend! After receiving the distressing news of Noah’s donor cell status, we learned that the military hospital back home does not feel that they can safely care for Noah when we return home. We appreciate their honesty and for seriously considering Noah’s welfare, but this puts a huge wrench into our plans. As well, we learned that the Army has placed Greg on orders for a duty station here in the mainland that we know for sure does not have the capabilities of caring for him either, with the nearest children’s hospital being 3 hours away…this is not safe and we definitely cannot go there. Fortunately, Greg’s unit is graciously trying to help us out with this issue and we are very grateful for how much they have helped us out during all of this, but those of you who know anything about trying to get orders changed, then you recognize the challenge here. We know that it will all work out, right? It always does somehow and we have learned to just go with the flow and try to figure out ‘why’ later when the dust settles. It is so amazing how this whole experience has completely turned our lives upside down and so often, but oh how dull our lives would be if we didn’t have such excitement. We are anxiously waiting for another chance at a donor cell test this week, desperate for a better result…praying, hoping, begging and pleading. We are also hoping to get the results back from his MRI too, but it may be too soon for this. They do a special diffusion study with his MRI that is relatively exclusive to Duke. They compare Noah’s brain myelination to that of several other kids his age, who were nontransplanted and I believe transplanted kids as well. This provides us with some idea of where he is and where he should be in comparison to the other kids. We are hoping that his numbers are up from his last one done in early June. Thank you ALL for the awesome uplifting support this weekend. It was a tough one, but we have nearly made it through and we are starting another week. We continue to thank God for all of you and your support, praying for all of the other transplant families, caring bridge families and of course our beloved Krabbe families!!! We are so blessed to have so many wonderful people in our lives, …family, friends, acquaintances and strangers alike, we are so grateful. Thank you Stephanie for the help and for the great conversation! I can’t wait to get to meet you guys next year in NY for the symposium. God bless.

Saturday, August 23, 2003 6:17 PM CDT

Entry 159—Aug 23, 03/Post Transplant Day 184/ Post Transplant Boost Day 122

All right, so we know we aren’t truly cursed…it was a nice release of steam. And we count our blessings every day, knowing full well that Noah could already be dead or at least without any donor cells left giving him an ultimate death sentence. We know that he has bounced back before. Things can always be worse…this we know too. However, we have been away from our home, our ‘life’ as we knew it now for over eight months. Noah’s life constantly remains in the balance, and we are exhausted beyond words. We can look around and see so many kids and families who had little to no complications or problems throughout their transplant journey, who are now making their happy way home at around their 100 day mark…and all I can think is, why can’t we be in that group and already be home with Noah doing well, fully engrafted and healing. There are others similar to us that have been here 8 months to a year, have spent their own time in PICU, and had numerous complications, so we are not the only ones suffering…but they are almost all engrafted to 100% despite their other issues. We don’t even have that. Noah is barely making enough enzyme to sustain the disease from progressing right now. For those who follow Noah’s progress continuously, I must ask this question, are we crazy in thinking that we have had a rougher time than the average person, or are we just delusional because we are in the midst of all of it? When bad things consistently happen, it does start to feel as though we are jinxed in some way, even though we do know that whatever happens is actually part of God’s plan and we have to just go with it. We will survive all of this no matter what happens, because it is in our characters…and we really have no other choice.
We know prayer works, and that God heals and provides miracles, we just wish that the prayers were working the way that we would like them to. Our beloved Pastor Gowen has talked with us many times about this, that the only promise God really gives is that He will never forsake us…this is the best promise that we could receive. It is hard at times for us to even see God in all of this. However, our human nature pushes us to greedily ask for more, for Noah’s life to be spared and for his complete healing. We wish that we could finally get some breaks, the ones that we have been hoping for anyhow. We aren’t complaining, we have been in tougher situations and things truly could be worse. I am just venting.

Noah had a really bad night, and we were worried that we might actually have to take him in to the PBMTU last night. He was on oxygen, needing suctioning about every ½ to 1 hour, and looked a bit in distress. We learned this morning that his albumin was really low…BINGO! His weight was up quite a bit today too. So we went in to have him infused and they gave him some extra diuretic. We are hoping that he will void a lot of this excess fluid out. He will have another donor cell test done Tuesday and we are hoping that his white blood cell count will be up high enough so that we can rule out any false readings due to it being too low. We are of course hoping that this past week’s result was due to the low WBC, but we will only know by taking another test this coming week. We hope to find out his MRI results this coming week too, that will tell us about his re-myelination status. Thank you for all of your great support and for trying to lift our spirits. We are fine, it is just hard on us to get bad news so often and we don’t respond as well as we used to. Still no word on when we will return home, but we don’t want to leave until we know it is safe for Noah and he is stable to be so far away from Dr. K who knows Noah so well. We are so very, very grateful to Greg’s command and my PICU unit at home for their understanding and support for us since we have been gone for so long. We can’t wait to get home an d we hope that this week brings better news. God bless.

Friday, August 22, 2003 9:55 AM CDT

Entry 158—Aug 22, 03/Post Transplant Day 183/ Post Transplant Boost Day 121

Okay, we are so done getting crapped on! Noah’s donor cell test came back today and he has dropped an entire 6%!!!!!!!! From 19% down to 13%!!!!!!!!! We can’t get a break…we must be cursed. We are too tired to deal with this, enough is enough!

Thursday, August 21, 2003 9:55 PM CDT

Entry 157—Aug 21, 03/Post Transplant Day 182/ Post Transplant Boost Day 120

Okay, so we didn’t get what we had hoped for with the medication changes and things didn’t go quite as we had planned in clinic today. It was yet another long night, up suctioning Noah, who required oxygen again to keep his saturations up above 90 Then this morning before taking him in to clinic he had saturations of 99n room air! Little stinker is being naughty! ;-) However, he is still much more congested than last week, but they are pretty certain it is just his regular mucous issues. They told us that this may just be part of his disease progression and not medications or treatments causing the mucous, but how come it is never constant, and it comes and goes so much? No one will know for sure until we get him off of some of these medications. The agreement today was that we would wait a few more days to see how Noah does. Since his old CMV med suppressed his white cell and platelet formation, they changed him to one that does not adversely effect the cells production. Noah has also been getting a medication called GCS-F every other day to stimulate white cell growth since his old CMV med suppressed the growth so much. Without this GCS-F he would not be able to make white cells. Since he is now on a new immunosuppressant that won’t adversely effect his cell growth, they want to stop his GCS-F and give him a chance to try and grow his white cells…and if he doesn’t then we may be able to assume that it could be that his major immunosuppressant dose is too high and causing his white cells not to grow. Does this make sense…it is quite confusing even to type it. We are again, hoping that his white cells won’t really grow and will stay low…this way it will prove that his immunosuppressant dose needs to be cut in half. Now it sounds ridiculous I suppose and we know that if we truly wanted to put our foot down with Dr. K about the issue, she would comply…, but we respect her opinions and her greatest fear is that Noah will lose the mere 19onor cells that he has and once that happens, we most likely won’t get them back and Noah will die from this horrid disease that he has fought so hard to survive. We would feel like total crap if we were wrong and she was right on this issue. We may get his donor cell test results back as early as tomorrow, but likely next week. We are eager to see if they have gone up any, and desperately praying that they have. May God have mercy on our baby Noah and heal him.
Tomorrow we are taking Noah to the Peds outpatient surgical clinic to have his G-Tube button replaced with a different brand. It may cause him some discomfort, but he should be fine. His button now leaks some and the extension always pulls out so easily that his stomach fluid starts pouring out…Yuck!! Anyhow, we hope it will work better. We will be drawing labs all weekend to follow up on his white cell counts to see his trend. Dr. K said that if it remains low despite the med changes, then she will likely consider dropping his immunosuppressant dose…finally. I hope this update was not all too confusing. We are praying for God’s blessings of mercy and healing for Noah. Thank you for your continued prayers and support.

I just wanted to add something else. I think that you are all aware of the big computer virus that has been causing us all such big headaches. Well, we have received numerous, numerous emails with the following subject headings which we believe are 'hot' with this virus and we have been deleting them immediately. I just wanted to list them in hopes of saving you all some headaches in trying to patch up your computers.
The subject headings have included:

'Re: Approved'
'Re: That Movie!'
'Re: Wicked Screensaver'
'Re: Details'
'Re: Welcome!'
'Thank You'
'Re:Re: My Details'
'Re: Your application'

We owe a huge thank you to Erika on the Krabbe message board for giving us the 'head's up' on this and we have not had any more problems with our computer since. I hope this helps.

Wednesday, August 20, 2003 7:10 PM CDT

Entry 156—Aug 20, 03/Post Transplant Day 181/ Post Transplant Boost Day 119

Well, back in clinic today to get his new CMV med, but a much shorter day…only 3 ½ hours. However, despite his white cell count only being at a low 3.3 (still up from yesterday’s 0.8), Dr. K wants to wait and see what tomorrow brings. Ugh! We were hoping to go ahead and be able to decrease his immunosuppressant today, but to no avail. We are again ironically hoping that tomorrow’s white cell count will be down from today so that she will be more likely to go ahead and cut his immunosuppressant dose in half. We think this will allow Noah’s donor cells to grow faster than they currently have been with little risk of graft vs. host disease. We simply want to at least try it and see. We will likely be back into clinic tomorrow too to hear what Dr. K has in mind for Noah’s meds. Last night was long, again spent mostly suctioning out Noah’s airway and he needed some oxygen. He was a bit better today, so hopefully it will be a better night tonight. Thank you for the prayers and support. God bless.

Tuesday, August 19, 2003 10:00 PM CDT

Entry 155—Aug 19, 03/Post Transplant Day 181/ Post Transplant Boost Day 118

Whew, what a LONG day!!! We just arrived home about half an hour ago (9:40pm) after arriving to clinic at 9:45 this morning! What a busy day though. Soon after arriving to clinic Noah received his 2-hour infusion of his CMV IVIG. Just in time to hear that his labs returned back showing that his platelets were low and he needs an infusion, and that his white blood cell count is down to a VERY low 0.8!!! Yikes!! They drew a donor cell test, but Dr. K isn’t sure that it is going to be very accurate if his white blood cell count truly was that low and not just a faulty lab result. So we might be shocked next week when we get his donor cell test back and have to redo it. By this time we had been in clinic already 3 hours and before we could do anything else we had to go to have Noah’s MRI done, which takes about 2 hours. After that was done we had to take him to have a lateral skull x-ray done under fluoroscopy to make sure that the MRI did not erase the setting on his electronic VA shunt in his brain. There was some question as to what the setting was originally, so we spent quite some time there and did not make it back up to the clinic Rainbow Day Hospital for Noah’s platelets until after 3 in the afternoon. He got his platelets and then we sat waiting for Dr. K…approximately 5 hours! She is one busy woman with a lot of kids to see, but the great thing about her is that she takes her time with each patient, giving them her entire attention…rather than hurrying through each patient just to get out at 5pm and possibly missing important info about her patients. She is very thorough, and we really don’t mind too much waiting to see her. It is the waiting on the days that we really don’t need to be there in clinic that get to be frustrating. Well, she made some more med changes for this week. We cut out one of his blood pressure meds completely! Yea!! We just have two more to go. She cut out his Erythromycin for gut motility, so we are down to just the Reglan. She is cutting both of his diuretics in half…, which is a bit scary given his history of fluid shifting issues, but we’ll see what happens. She is changing his CMV med to a different one, which will be less suppressive to his platelet and white blood cell count production. She is also considering cutting the Cellcept, his major immunosuppressant, in half!!! This is the med that we think could be contributing to his fluid problems and maybe even affecting his vision…and we attempted to convince her to reduce it last week, but she didn’t want to. We will check Noah’s white cell count in the morning and we are kind of praying that it will be low, ironically, so that maybe this will allow his donor cells to grow better and we can see if it helps his mucous issues & vision. She did not touch his methadone or steroids, which we were hoping for, but maybe next week. Before leaving, we asked her about something that we noticed this morning. Noah’s right arm is more swollen than his left and she said that it might likely be a small clot in his vein and to monitor it tonight and in the morning. If so, he might need an ultrasound tomorrow. Ugh! Well, that was our day in a nutshell. We are to go into clinic tomorrow to receive the first IV dose of his new CMV medication to make sure he doesn’t have any reactions. Hopefully it will be a much shorter day for us. We thank you for the prayers and support. We ask that you will say some extra special prayers for a few kids/families that are in great need of some extra blessings. Manny and his family need a lot of prayers right now as they have recently been confronted with some very difficult decisions.
www.caringbridge.org/sc/mannymiramontes

Ashleigh, who is starting the transplant process, but still suffering the symptoms of her Krabbe, is in need of prayer. We can personally empathize with her parents, as Noah suffered symptoms from the day we brought him home, screaming and crying inconsolably for about 20 or more hours a day…and this is so VERY, VERY trying on one’s patience and very frustrating. So we ask that you offer your encouraging support and prayers for them as well.
www.caringbridge.org/nj/ashleigh.ryann

Monday, August 18, 2003 0:50 AM CDT

Entry 154—Aug 18, 03/Post Transplant Day 180/ Post Transplant Boost Day 117

Wow, already to day 180 and still here at Duke! Well, we could be in worse places and in worse situations, couldn’t we?? First and foremost, we want to say a HUGE ‘MAHALO’ to a new friend who generously raised money for Noah by having a yard sale on Saturday! Thank you so very much Susan!! What a blessed heart full of love and kindness lies within you, and we are truly blessed to know you and we appreciate your gifts so very much! God bless you! This money will be helpful in paying for Noah’s prescription co-pays, sterile water and supplies for him! But, Thank you also for obtaining your Hunter’s Hope packet to become an official H.H. volunteer!!!! On behalf of the entire Krabbe Family, we say THANK YOU!!! Kelly can definitely use the help here in Durham to help all of the Krabbe families that come to see Dr. K at Duke for transplant. Maybe we will even see you next year in NY at the Hunter’s Hope symposium!!! We are so delighted that we were able to meet you personally yesterday, and that you were able to personally see Noah…as not many have, we keep him hibernated away to hopefully reduce any risks and prevent infections. ;-) We owe a much deserved and much belated Thank you to Deanna Climer and friend for the care package we received several weeks ago!!!! Thank you so much! As well, Thanks Dad and Esther! We love and appreciate you both for the tremendous support that you have shown us during this entire ordeal. Thanks mom for working so hard to increase public awareness about Noah’s disease and other leukodystrophies. Best of luck at the upcoming sale!
Noah has been doing well…knock on wood…and we are setting a record this week by not having to go to clinic for a week!!! Go Noah, Go Noah!!! He remains relatively the same, mucous issues are moderate, but he has not had any need for oxygen, which is great! We are anxious for the tests this week…but we have become experts at receiving bad news, which means we have become difficult to surprise…unless it is unexpected pleasant surprises! And that is what we are praying for!!! Increased donor cells, increased enzyme level, and an increase in his re-myelination! We are hoping this week that Dr. K will make some more medication changes such as weaning him completely off of the methadone, weaning his steroids down, and hopefully weaning him off of one of his three high blood pressure medications. Ever since he was placed on high dose steroids in the PICU back in May/June, his blood pressure has been way too high as a bad side effect and he has needed a total of three meds to keep it within a normal range. Well, as he has been weaned off of the steroids we have been able to cut one of them in half, but not wean it completely. We are hoping that this week we can finally wean him off of one completely! We are moving closer to getting home…but have not been given an actual date yet. We are crossing our fingers for the first or second week in September! Given that it is already the third week in August, we are not sure this will happen, but again we are praying! Thank you to those who diligently pray for Noah’s complete healing each day! We are indebted forever for your support and kindness; for your prayers for our son’s life are priceless indeed! Thank you and God bless!

THANK YOU, GOD, FOR EVERYTHING!

Thank you, God, for everything—
the big things and the small,
For “every good gift comes from God”—
the giver of them all—
And all too often we accept
without any thanks or praise
The gifts God sends as blessings
each day in many ways,
And so at this ‘Thanksgiving’ time
we offer up a prayer
To thank You, God, for giving us
a lot more than our share…
First, thank You for the little things
that often come our way,
The things we take for granted
but don’t mention when we pray,
The unexpected courtesy,
the thoughtful, kindly deed,
A hand reached out to help us
in the time of sudden need…
Oh, make us more aware, dear God,
of little daily graces
That come to us with “sweet surprise”
from never-dreamed-of places—
Then, thank You for the “Miracles”
we are much too blind to see.
And give us new awareness
of our many gifts from Thee.

—Helen Steiner Rice

We are so very blessed; blessed far beyond what we could have ever imagined. We were dealt cards that have been difficult to handle, but God also dealt us the love, support, and strength ‘cards’ that we need to play out the worst and survive the ‘bad hand’. We have come to try and not overlook, but appreciate even the smallest of miracles and blessings graced us by God. Each of you have been brought in to our lives for a reason, and although we may never personally meet you, we know that you are there by the encouragement you provide us… and we thank God for you! Praise God for these blessings! We are truly blessed!

Saturday, August 16, 2003 6:05 AM CDT

Entry 153—Aug 16, 03/Post Transplant Day 178/ Post Transplant Boost Day 115

HAPPY 11 MONTHS BIRTHDAY NOAH!!!!!!! What an accomplishment, especially with this disease! Yea Noah!!! Next month’s will be a huge celebration! I am not normally up this early, I absolutely, positively do not do mornings at all…I am a complete night owl; but since Noah’s lab draws, meds, tube feeding, and respiratory treatments are due…here I am! He had a great night, Praise be to God!!!! His oxygen saturation was 98-100% on room air! Now I hesitate to rejoice too much, because it often seems that this mucous thing goes in waves and comes just as quickly as it goes, but we will rejoice anyhow for the sleep that we received, and for the break from clinic that we have been having lately. We have not been since Tuesday, and if his labs are fine today, which we are expecting them to be, then we don’t have to go in either…not until Tuesday! Wow! Six days off between clinic visits, that is terrific! I guess though, at almost 200 days post transplant that Noah should be able to go to clinic once a week, but given all of his extensive complications inpatient, it is a small miracle! Yea!!!
We had a great visit yesterday with the Forslind family, an extremely sweet Krabbe family that is here for Kyle’s post transplant check up, and we want to thank them for dropping in and bringing us some gifts! Thank you Dave, Fran and Kyle…we will pray for your safe trip back home and Kyle’s continued recovery!
Thank you all for your continued support and prayers for Noah, and for all of the other kids/families! We appreciate it so much, and Noah seems to be making minute advances in his recovery and we are very grateful! We are praying for his complete healing…there is a lot to be done to get there, but God is able and we are praying for his blessings. Thank you for those who went to Daryn’s and Dylan’s web sites to offer support. These are a couple of Krabbe boys that need some support. God bless.

www.caringbridge.org/mi/darynj

www.caringbridge.org/europe/dylan

www.caringbridge.org/nj/ashleigh.ryann

www.caringbridge.org/il/elijahbarron

www.caringbridge.org/co/deganmiles

Friday, August 15, 2003 8:15 AM CDT

Entry 152—Aug 15, 03/Post Transplant Day 177/ Post Transplant Boost Day 114

Well we had another night of a little sleep. Yea!! We are getting spoiled! Noah has still had some issues with his mucous, but not like it has been earlier this week, although we are not holding our breath since this mucous problem seems to come and go so fast. We did not have to go to clinic yesterday since Noah’s labs were all right, so we were able to cat nap too. What a treat! Anyone recall the prayers for poo that we needed way back in early May? Well, no problem in that department! Noah has had enough to last a lifetime the last few days! ;-) We are grateful that everything seems to be working in that department… although he is still on two medications to keep his gut moving, which seem to definitely be doing their job. Just thought I share that there are some things that are going well for Noah. We don’t have clinic today…so we will spend our day trying to relax a bit between all of Noah’s care. We want to mention our appreciation for the kind generosity from a family by the last name of Smith from Texas for your gracious gift! We are so very grateful and God bless you for your kindness! We could never begin to tell you how much this means to us! Thank you!
We also wanted to mention that our Krabbe buddy, Daryn, finally has a web page that has been set up, but it is still in the works. However, we are asking everyone to stop in and sign his guest book, offering them encouragement and support. Daryn has late onset Krabbe, is three (I think), and is now on the PBMTU just out about a month or so of transplant.
www.caringbridge.org/mi/darynj

We appreciate all of your support and prayers! We are eager to get his testing started next week, and even more anxious to get the results. We continue to keep all of our fellow PBMTU kids/families, Krabbe kids/families, and Caringbridge kids/families in our prayers! There are so many prayer requests among them all. God bless.

Thursday, August 14, 2003 9:02 AM CDT

Entry 151—Aug 14, 03/Post Transplant Day 176/ Post Transplant Boost Day 113

Tuesday was another long night, but last night was fair…not too bad. We actually got a few hours of sleep! Yea!! We went to clinic yesterday because his lines would not draw, so we had to go in to let them draw blood, give platelets and put TPa into his line to break up any clots that may be there causing them not to draw. With a bit of effort, they finally drew today and we have not yet heard if we need to go in to clinic for anything. We are waiting for Noah’s PNP to call us and let us know. Noah is still doing about the same. We have not yet received the schedule for his tests that are supposed to be next week, but we are eagerly waiting to hear. We are most anxious to see the results of his donor cell and enzyme tests and his MRI, which will tell us if he has myelinated any more than the last MRI that was done back in early June. It has not been a long time since the last one, but we are hoping that we will see more re-myelination (and donor cells & enzyme too).
We want to thank you for your continued support and prayers. Manny, our PBMTU friend, is getting discharged this week!!!! Maybe tomorrow? Please go visit his page and offer him and his family support! Yea Manny!!!! We would also like to offer our condolences to a PBMTU family whose son passed away yesterday in the PICU. Our prayers are with them during this very difficult time. There are so many kids and families that need prayer. God bless them.
www.caringbridge.org/sc/mannymiramontes

Wednesday, August 13, 2003 2:16 PM CDT

Entry 150—Aug 12, 03/Post Transplant Day 174/ Post Transplant Boost Day 111

Whew, after a nine hour clinic day for just a two hour infusion, we are so exhausted! Noah’s labs were fine and he didn’t need any blood today, but probably tomorrow. We had our discussion with Dr. K for some time at Noah’s bed side, and she listen thoroughly to our point of view; but explained that there is no need to give Noah the immunosuppressant at a lower dose because it would not be kept at a therapeutic level…and basically useless to even give any of it. She said that it is not worth taking the risk of his host cells killing off his donor cells or him developing graft vs. host disease. Her main priorities are to protect the donor cells he has, foster their growth, and prevent him from getting graft vs. host disease. She gave us no estimate as to when we can return, but Noah’s six month post transplant studies are next week, which consist of an MRI, ECHO, EEG, Donor cell blood test, enzyme test, and immunity test. With all of this we will be able to see if he is continuing to remyelinate, if he is still seizure free, if his heart is still functioning normal, if his donor cells have increased from the previous level of 19%, and at what level his enzyme is. We did make some changes though, she did reduce his methadone again, and he should be completely weaned off of that in a couple more weeks. As well, she decreased his steroids dose, so that we can eventually wean him off of these in a few more weeks too. We did not argue too much with the whole immunosuppressant issue, as we don’t want to put Noah into harm’s way and Dr. K is much more experienced at this stuff than we are, but it was worth a try. She did say that since she was out of town for a week and has not seen him for two weeks, that Noah was looking much better to her. This is good to hear. We continue to pray for his healing. God bless.

Monday, August 11, 2003 4:30 PM CDT

Entry 149—Aug 11, 03/ Post Transplant Day 173/Post Transplant Boost Day 110

Noah gave us another rough night, so much mucous it was foaming out of his mouth, he was desaturating and we put him back on the oxygen. Dear God I wish we could figure out what it is that is causing him to have this problem. It is getting to be too much. We spent our night hours suctioning him again, purely exhausted this morning. We spoke with Noah’s PNP at the clinic about doing another donor cell test, reducing the dose of his immunosuppressant and waiting 2-3 weeks to see what happens on the lower dose…there could be a few things that we could see. One, no changes, he has no reaction either way…good or bad. Two, he could develop Graft vs. Host, where on the lesser doses of immunosuppressant, the donor cells are ‘less’ suppressed and are able to go a bit hog wild, thus they begin to attack his body…which can be very dangerous if it attacks major organs and gets out of control. Third, it could be just enough of a reduction that it allows his donor cells room to grow more and at a faster rate than they have been growing, and this is of course the one we are looking for. As well, though, by reducing his dose we are hoping that he will have less of a problem with this mucous. If it is this medication, then it probably won’t matter what dose amount he is on, because if he has developed a sensitivity to it, then he will probably have a reaction as long as he is getting the medication into his system. However, we are gonna try. This is getting to be old, we are so very tired, and it is at least worth trying to find out if it is this med. Now, the only thing we have to do is convince Dr. K that this is a good thing, and that we should give it a try. For those of you who know her, this won’t be easy, but she has been flexible at times so we are hoping that she will allow us to at least try it. We know that if she disagrees, it is only because she is worried that it could harm Noah, but his lungs filling with fluid and his airway blocked by excessive mucous is dangerous too. We are crossing our fingers that we can find out the cause and SOON.
His labs were all funny this morning, so we think it was just a bad sample. His lines are still giving us a hard time when we try to draw blood from them. Our big clinic day is tomorrow, so we will have to wait and see what is in store for him. Thank you so much for the continued support and prayers. We certainly are in need of them. This mystery has to be solved before it puts Noah back into the hospital. We also ask that you will continue to remember the other kids/families who are suffering from these terrible diseases, and for the parents who have lost their children to these diseases. We cannot imagine getting through these last several months without all of the prayers.
Thank you so much for the wonderful compliments on the paintings. We fueled our emotions into those paintings, emotions that have compounded over the last several months from some extremely difficult events in our lives…they tell a piece of our story and the pain that has filled it. A candid look at our pain…
God bless you.

Saturday, August 9, 2003 9:16 PM CDT

Entry 148—Aug 9, 03/ Post Transplant Day 171/ Post Transplant Boost Day 108

Noah has been relatively the same…no real changes in the last day or so. We spent a lot of time today catching up on lost sleep. It seems that is all we do in between going to clinic and performing Noah’s daily routine of care. Since we did not have to go to clinic today, then we spent some time trying to positively fuel some of our pent up emotion into painting today. Upon request, we took some digital photos of some of our paintings and decided to post a couple of the photos in the photo album, one by Greg and the other mine. I was once told that a person’s creativity is at its most heightened point when the person is extremely tired…seems to be true for us.
We will draw labs in the morning and if Noah is still doing good, then we most likely won’t have to go to clinic tomorrow. We are hoping that it will be another day to stay home.
Thank you for all of the prayers that are seeing us through this difficult time. We ask that you will continue to remember Cody, Ashleigh, Dylan, Daryn, RachelJoy, Cheyenne, Degan, Elijah & Alissa, Madi, Nolan, Jordan, Manny and so many, many more of the transplant kids, Krabbe kids, Caringbridge kids and those parents who are now without their kids. God bless them all.
www.forcody.org
www.caringbridge.org/sc/mannymiramontes
www.caringbridge.org/nj/ashleigh.ryann
www.caringbridge.org/europe/dylan
www.caringbridge.org/co/degan
www.caringbridge.org/il/elijahbarron
www.caringbridge.org/nc/princessmadison
www.caringbridge.org/co/nolanramsey
www.caringbridge.org/il/jordan
www.caringbridge.org/or/racheljoy
www.caringbridge.org/tx/cheyenne5

Friday, August 8, 2003 3:42 PM CDT

Entry 147—Aug 8, 03/ Post Transplant Day 170/ Post Transplant Boost Day 107

It was a better night, not on oxygen, but still sounding quite junky in his lungs. We drew his labs early this morning and dad dropped them off, then returned to help finish up his morning meds, and then we went back to sleep. We are getting so worn out, always feeling like we are fighting an endless battle to keep our son alive and with us. We did not go into clinic today, just called for his lab results, spoke with the PNP about our night, and decided just to keep a close eye on him and call to bring him in if necessary. Keep a close eye on him…hmmm, how much closer an eye on him can we keep? We are in a much better situation now than we were in the past, but our overall journey has been so tough. We appreciate and are blessed to have him alive and ‘home’ with us…but is it greedy to say that it is not near enough? If we were not staying up all hours of the night and day, watching him like a hawk, suctioning him every 20-30 minutes…he would be inpatient, maybe dead already. We know that it was our choice to keep fighting for him, and it is still our choice…but we wish that it wasn’t so hard all of the time. We keep praying that God will allow us to have some relief, either clearing up Noah’s mucous/airway problems or leading us to the cause of the problem so that we can try to fix it ourselves. It is amazing that we arrived here with a son who was able to see, hear, smile, coo, laugh, suck, swallow, cough, gag, move his arms, hands, legs, feet, head, and had relatively no medical issues/needs despite his severe irritability from his disease…and we now have a child who most likely cannot see, hearing…possibly, does not smile, laugh or coo, can no longer suck, swallow, cough or gag effectively, and only weakly moves his arms, hands, legs, feet, and head. This may be a case of ‘glass is half full or half empty’, but I don’t know of anyone who can stay optimistic all of the time. We are grateful for Noah’s life being spared and that he is starting to make some weak movements in his extremities. We are grateful that Noah’s donor cells are starting to slowly grow back…but why did he have to lose them in the first place? The questions will remain unanswered, and we will continue to take life day by day. We know that our future is uncertain, and that great things may be in store…we are hoping and praying for them. We know that many of the Krabbe kids who have lost a lot of their functions, are getting them back after remyelination begins to take place…and Noah is starting to remyelinate. Kids that were once blind are now able to see things…how well they see is still uncertain, but they are tracking objects now. Kids that were once in Kid Carts are now starting to walk and ride tricycles. We ask that you will continue to keep Noah in your prayers, and us too, that we will be given a break. We also want to mention a good friend, Cody, who is quite sick right now and needs some extra prayers. He is in the PICU at his home, and will be transferred here to Duke around Monday, but we ask that you would remember him in your prayers too.
www.forcody.org
Thank you so much and God bless.

Thursday, August 7, 2003 6:30 PM CDT

Entry 146—Aug 7, 03/ Post Transplant Day 169/ Post Transplant Boost Day 106

Noah again kept us up almost all of the night with his increased mucous problems. He began to desaturate last evening and needed oxygen to keep his oxygen saturations in the 90s. Despite our best efforts, his lungs still remained junky, and we dragged ourselves in early to clinic to get some help. Noah’s main PNP was there to meet us in the hallway and shuffled us back to a room in the Rainbow Day Hospital. Noah had a respiratory viral battery test done, testing for any virus that may be in his airway. We then took him to have a chest x-ray done, and it showed a possible pneumonia in his right upper lung. He has been started on another antibiotic just in case it really is pneumonia and not just some atelectasis…he has already had allergic reactions to 2 other antibiotics. We are hoping that it is just some minor atelectasis and not infection. He has had no fevers and today, in clinic, he no longer needed the oxygen and began to have better oxygen sats…having a lot less mucous. We are all so very, very puzzled as to why this is happening. His inhaled steroid was increased as well, so maybe this will help. It is such a big mystery as to what is causing him to have such issues with mucous, but it comes and goes so frequently. We are all thinking that it could possibly be a medication, but not sure which one, or if it is a combination. However, most of the meds he is on right now, he needs to be on and cannot be weaned from. So this leaves us in a difficult spot. We will have to wait until Dr. K returns from out of town and speak with her next Tuesday… hoping that Noah does all right through the weekend.
We ran into Degan and his parents in clinic. They were just discharged on Tuesday, and are getting a huge taste of what clinic life is like. We offer our support to them and are keeping them in our prayers. (www.caringbridge.org/co/deganmiles)
We also were able to meet Ashleigh and her parents today. She is recovering well from her abdominal incision infection, and will have surgery next Tuesday for her central IV lines to be placed. On Wednesday she will begin chemo. We are praying for them as well. What a very beautiful girl she is! (www.caringbridge.org/nj/ashleigh.ryann)
Princess Madi is being released today from the unit after being treated for her central IV line infections, and we continue to pray that she remains infection free and that she kicks her cancer to the curb! Go Madi!! (www.caringbridge.org/nc/princessmadison)
We made a stop in to see our good friends on 5200/PBMTU, and it was so nice to see so many familiar faces (just to visit…). We do miss seeing them and we are so glad we came up to the unit, despite being so tired today. Congratulations Bobbie on your much earned nursing accomplishment!! You certainly deserve it! You all (5200) deserve an award! You have taken such great care of our entire family, and have a special place in our hearts.
I just wanted to wish my good friend, Betsy, a very Happy Birthday tomorrow!
Thank you for all of the continued support and prayers! We are so very, very grateful. God bless!

Tuesday, August 5, 2003 8:15 PM CDT

Entry 145—Aug 5, 03/ Post Transplant Day 167/ Post Transplant Boost Day 104

Today was our big clinic day and it went well. Noah held on to his hemoglobin through today, but was at his cut off today and so he received some blood. Not bad though, 14 days! Dr. K is out of town, so Dr. S saw us today and all went well. Some changes were made in his medication regimen in hopes of trying to stop this mucous problem. His weight was still up quite a bit, and last night he was really bad again, needing a lot of suctioning and his pulse oximeter was beeping a lot too. We are hoping that these changes will do the trick so that we can finally be done with this problem. Noah is still making some small movements with his head, arms/hands, and not legs/feet…but still seems quite sleepy most of the time, and we are still questioning his ability to see, praying that his sight will be restored. We are almost weaned off of the Methadone, and we are going to try and wean him off of the Benadryl this week. Hopefully, this will allow him to wake up some more. Noah was fitted to his Kid Cart today!! He looks absolutely adorable in it, and seems to be quite comfortable in it. For those of you who knew Noah before transplant, his disease caused him to posture by arching his back a great deal and in doing so, he would get even more agitated and upset being strapped into a car seat or stroller…screaming the entire time he was harnessed in. Car rides were absolutely torture for the entire family…kind of ironic that was what everyone was suggesting that we do, since they were certain that he had colic, but Noah did not like car rides at all! Well, I was a bit worried with strapping him, but he did fine and has been sitting in it here at the apartment for a couple of hours, and has done fine! It provides him some great support and we are so thankful to our fellow Krabbe families who recommended it. We will post new pictures tonight…finally! It is a long story, but the technical difficulties have been fixed and we are up and running again.
We were able to meet little miss Laura, a fellow Krabbe kid, and her mother Carol. They are here to have some post transplant studies done, and they stopped in today to visit. We were so very delighted! Please see her web page below to read about her story! She is a cutie! Thanks for stopping in guys!
I made it up to the PBMTU, newly renovated, and was able to see baby Degan (another Krabbe kid, newly transplanted) make his jailbreak today! Yea Degan!!!! We wish them the very best in outpatient and hope to see them in clinic this week! Please visit their web page to wish them well; it is listed below!
We want to make some special prayer requests before closing this journal. Princess Madi is inpatient with central line infections…needs a lot of prayers.
Cory, who had an unexplained seizure while in outpatient, has now been admitted for observation and to find the source of his recent episode. Please keep him in your prayers. He is such a sweet kid. Daryn, a fellow Krabbe kid inpatient and recently transplanted, needs a lot of prayer. He does not have a web page right now, but we wanted to make mention of his prayer need. Ashleigh, a Krabbe kid who was supposed to start chemo this week, was admitted for an infection found around her GT site (which was surgically placed a week or so ago). She was unable to get her central lines placed in surgery today after they discovered the infectious areas in her abdomen, and now her chemo/transplant is now postponed. She needs a lot of prayer right now, too. Dylan, another Krabbe kid getting ready for transplant, had his shunt placed today…just like the one that Noah had placed. We do not have a web page for them yet, but hope to find out if they have one soon. Thank you prayer warriors, for all of the tremendous help in sending up prayers for Noah and all of the kids. Prayer works, and we need it…a lot! Thank you and God bless.
Laura www.caringbridge.org/mo/lauranews

Baby Degan www.caringbridge.org/co/deganmiles

Madi www.caringbridge.org/nc/princessmadison

Cory www.caringbridge.org/va/cory.bishop

Ashleigh www.caringbridge.org/nj/ashleigh.ryann

Harmen www.caringbridge.org/europe/harmen

Cheyenne www.caringbridge.org/tx/cheyenne5

Elijah & Alissa www.caringbridge.org/il/elijahbarron

Manny www.caringbridge.org/sc/mannymiramontes

Sunday, August 3, 2003 6:26 PM CDT

Entry—Aug 3, 03/ Post Transplant Day 165/ Post Transplant Boost Day 102

Wow, two days in a row…I must have some time on my hands! Well, Noah did have a better night, and thus that meant that WE had a better night. As well, Noah held onto his hemoglobin and did not need transfusions!!! Praise God for this answered prayer! We suspect he will need them by Tuesday, his next clinic day, but even if this is the case, he has at least gone 14 days since his last transfusion!!!!!! Yea Noah!!!! Thank you God for this blessing…we will gladly rejoice in it and celebrate! We continue to pray that he cranks out those red blood cells and platelets, so that we can hurry back home soon. And we continue to pray for donor cell growth to keep on going!! We hope that he will at least be in the low 20’s next time!! Dr. K said that once Noah’s donor cell percentage reaches about 50%, that we can then back off on his major immunosuppressant medication, which might help his donor cells to be able to grow at a little faster rate…and he would be at less risk for ‘Graft vs. Host Disease’…when the donor cells recognize his body as foreign and start to attack it in a bad way. For right now, he remains on this drug, so his donor cell growth rate is slow, BUT it is rising!!!!!!!!!!!!!!!! YEA NOAH!!!!!!!!!!!!!! He has had what seems to be less of a problem with mucous the last 24 hours, why? We don’t know, but we are in the works of trying to figure it out. We will skip his Cytogham dose this week (his antibody infusion) to see if maybe this is the cause, but Dr. K doesn’t really think it is…but the great thing is that she is quite willing to help us figure out what is causing the problem and has been very flexible in his medication regimen. We hope to get his Kid Cart this week. It is a cross between a stroller and a wheelchair, and they are very nice! We are so blessed to get one! It will be much heavier to get in and out of the trunk of the car, but it will be better for Noah. We are excited. Kelly has loaned us her son’s Panda Seat (?), which is very similar to a Kid Cart, to try out. Thank you Kelly! We have been enjoying the days away from clinic, three days in a row! Thank you God! We have been utilizing our extra time (????what is that???) and energy into expressing our creativity…painting, Dad with acrylics and Mom with her watercolors. Dad is the artist, mom is an amateur trying to experiment and pick up some of his techniques, but it has certainly been a great release. We hope to one day allow Noah to express himself through art…macaroni art on Dixie plates, finger paints, who knows what he can do. Thank you for the continued prayers, please know that some of them have been answered and we rejoice with Thanksgiving! God has been so good to us, and we are truly blessed! Thank you for remembering all of our PBMTU kids/families, Krabbe kids/families, CaringBridge kids/families, and those parents who have lost their children. God bless them all. Again, we offer some web pages of kids/families in need of prayer. Thank you to those who have been so very faithful in visiting these web pages and offering your support, encouragement and prayers on behalf of these kids/families. They each have their own unique story to share, and there is so much about life to be learned by each child/family! God bless.

Caleb www.geocities.com/calebwaddell

Rachel www.caringbridge.org/or/racheljoy

Samantha www.caringbridge.org/ma/samanthatherese

Jacob www.caringbridge.org/wi/jacobdavid

Ryan www.caringbridge.org/ct/oursonryan

Andrew www.caringbridge.org/page/andrewshope

Laura www.caringbridge.org/mo/lauranews

Gina www.caringbridge.org/ky/ginaswebsite

Harmen www.caringbridge.org/europe/harmen

Noah www.caringbridge.org/tx/noahsjourney

Saturday, August 2, 2003 4:24 PM CDT

Entry 143—Aug 2, 03/ Post Transplant Day 164/ Post Transplant Boost Day 101

Noah is keeping us busy, depriving us all of sleep, but hanging in there. He is still having issues with a large amount of mucous in his airway, and it has kept us up the last two nights trying to keep his airway clear enough that he can breathe without the oxygen. It is difficult for us to relax enough to try and get to sleep when we lie next to Noah listening to that mucous in his airway, at times sounding as if he is drowning. We also have to endure his withdrawals. There is nothing quite like enduring a Krabbe kid having withdrawals…but we are getting by, holding out for better days. We are so very, very tired right now. We have been going to clinic on what were supposed to be ‘stay at home’ days much more often now, usually for little things, but because we spend so much time sitting and waiting around, these days turn into all day events. I know that the other outpatient families can certainly relate. A good example of this was our 10-hour clinic day just to have blood drawn from Noah’s central lines so that we could find out how his blood counts and chemistries were doing. We know that it is in Noah’s best interest, so we try not to complain much, but it is getting to resemble how things were at home in Hawaii when Noah was not yet diagnosed and we were up all days and nights with him crying inconsolably…all of us exhausted. He is not crying as he used too, Thank God for that, but he is requiring a lot of extra attention. We think that there may be a medication that is causing him to have a sort of reaction, leading to this mucous issue. It comes and goes, and at times he is relatively clear with oxygen saturations of 99-100% on room air! We are praying that we can figure out what it is that seems to be irritating Noah’s airway and causing him to have so much mucous.
We went into clinic yesterday as well to get Albumin. We did not have to go today, which is actually a normal clinic day for us, but we will go tomorrow. Noah looks quite pale today, very ashen, so we are guessing that his hemoglobin is low and he will need some packed red blood cells tomorrow. He has not had any red cells infused for 11 days now. We are so grateful that he is holding on to these cells better than he had been, requiring transfusions less often. We appreciate all of your support and prayers, they are certainly needed. I am listing below some web pages of our fellow PBMTU, Krabbe and Caringbridge friends/family. It is always nice to read encouraging guest book entries, and I know that some of you are visiting these sites and signing their books. I certainly appreciate it, and I know that these kids/families do also. Thank you. Please continue to visit these sites and offer your support and prayers. And to those of you who stop in to visit Noah’s page and maybe even the others that are listed, but don’t sign in, we appreciate you too! Every prayer and thought matters, and God bless you!
Madi www.caringbridge.org/nc/princessmadison

Degan www.caringbridge.org/co/deganmiles

Ashleigh www.caringbridge.org/nj/ashleigh.ryann

Jordan www.caringbridge.org/il/jordan

Brent www.caringbridge.org/ga/brentr

Jeremiah www.caringbridge.org/ia/jeremiah

Nolan www.caringbridge.org/co/nolanramsey

Alex www.alexupdate.com

Katia www.caringbridge.org/fl/katia

Zoe www.caringbridge.org/il/zoejowolsfeld

We have a huge list of Caringbridge sites and web pages listed on our ‘Favorites’, of kids and families who really need as much if not more prayer as Noah right now…who are enduring their own difficult journeys and need encouragement. I feel that it is important to share their needs with our friends, families, prayer warriors and supporters that visit Noah’s page. That is why we always list them with journals. It is reaching out to others who have reached out to us…we are all a part of God’s family, and it is by helping each other that we will get through these rough times, and they are definitely rough!

Thursday, July 31, 2003 3:56 PM CDT

Entry 142—July 31, 03/ Post Transplant Day 162/ Post Transplant Boost Day 99

It is hard to believe that it has already been 4 days since the last journal entry…we are slacking, but our exhaustion would suggest otherwise! We have been keeping busy with Noah’s care, but he has been busy too, growing donor cells. His last Donor cell test taken last week returned, and he is now at 19!! This is up from the 16.5 few weeks ago! YEA NOAH!!! Grow cells Grow!!! Thank you for the ‘cell growing’ prayers!! Dr. K said that she believed his donor cells would only continue to grow, BUT…we all know that Noah had donor cells and lost them, and there is always a chance for either this to happen again, or for him to reach a certain point and then not grow any more. Since he has a metabolic disease and not leukemia, then it would be alright for him to keep some of his old cells, just as long as he has some of the new donor cells to make enough enzyme that he needs to prevent further damage. However, we are aiming and praying for 100ngraftment!!! We praise God for the 2.5hat he has made in the last few weeks!!
Overall, Noah is doing relatively the same, having a few bumps every now and then. We are in clinic about 4-5 times a week instead of the projected 3 days a week, but whatever it takes to keep him outpatient with us. He is still having some issues with mucous in his airway and inability to clear it, his fluid shifting a bit, and blood infusions. However, his infusions are getting farther apart, meaning that he is slowly starting to grow his own red blood cells and platelets. He is now running about 10 days between red blood cell infusions, and about 7 days between platelet infusions. Not bad at all, and we are praising God that it is not everyday. We know that there are many other kids that come to clinic a lot more often for infusions. However, for us to be able to go home, Noah needs to be making more blood cells on his own than he is right now. Please continue to keep him in your prayers, for his donor cells and red blood cells/platelets to keep growing, for him to continue to wake up and move more, and his eyesight to return (we are still not certain that he can see, his eyes remain rolled up or to the side a lot and he does not track objects). We are so very grateful for all of the prayers and support that we have received from so many of you…we have been pleasantly overwhelmed and touched by the amazing love and support we have received. The guest book messages that you leave are so uplifting and wonderful to read. Thank you!
We want to personally say Thank you to those of you who visited Ignacio’s web page and offered words of encouragement to his family. God bless you for the kindness that you have shown! We again are requesting prayer for all of the PBMTU kids inpatient, outpatient, and those at their real homes, the Krabbe kids/families, and those who are now without their children. As well, we want to make special mention of a few that need some extra prayers, and ask that you would reach out to them and visit their web pages and sign their guest books offering them encouragement.
Luke www.caringbridge.org/oh/lukeervin
Zoe www.caringbridge.org/il/zoejowolsfeld
Madison www.caringbridge.org/nc/princessmadison
Manny www.caringbridge.org/sc/mannymiramontes
Alex www.alexupdate.com
Ashleigh www.caringbridge.org/nj/ashleigh.ryann

There are many, many, many more wonderful and very special children that need a lot of prayers and we continue to keep them in our prayers. These are some needs that have been brought to our attention, and we wanted to utilize Noah’s journal to not only give updates of his progress, as a forum for sharing our thoughts and feelings (a.k.a. venting), but also for sharing news and the needs of others. We are so very grateful to those of you who have taken the extra time out to not only show your support for Noah, but for these other children that we have made mention of here on his page. I have noticed, when signing these other guest books myself, the familiar names offering wonderful encouragement. Thank you! I know how much it means to these children and their families! God bless you!!!

Monday, July 28, 2003 1:08 AM CDT

Entry 141—July 27, 03/ Post Transplant 158/ Post Transplant Boost Day 95

I cannot imagine how much more heartbreak my heart can withstand, and I speak not of my personal experience, but it is still terrible even from afar. Ignacio has passed away. Another sweet young and innocent soul has gone from this earth, taken from the loving arms of his family, leaving them with emptiness and heartache. True, for the believer in the precious gift of eternal life, life after death, then it should be a good thing, but my human heart and spirit have become so broken with all of the anguish and despair that is placed into the laps of families who have lost their children here. I suppose in the ‘real world’, where so many of you are living and a place I used to long for, the death of a child is a rather rare occurrence, but here in ‘our world’, it has become an all too often slap in the face of reality and just proves how much life can truly suck sometimes. In my line of work, what was once my ‘real world’, I experienced child death more often than the average person, but it was so very different. You see, when the children came into my unit, there was no opportunity to really get more than acquainted. It is my work, my job, and my mind is set in a completely different mode…emotions put aside and replaced with critical thinking about the patient and the tasks at hand. Often times I became so desensitized and totally mechanical…’feeling pity’ for the child and family standing by, but concentrating on hanging drips, medications, doctor’s orders, vent settings, vital signs, etc. It is so amazing to me now just how insensitive and cold I was, but I don’t think I was alone. I suppose though, that if I was too emotional then I would have been a blubbering mess and unable to successfully care for the patients. It was shamefully too easy at times for me to internally whisper my opinions that the parents crying at the bedside should call it quits already with their child, who was barely hanging on with life support and would only return to life with a miracle…and those are few and far between. Just offering the best words of encouragement that I could, I would step around them to grab another set of vital signs, calculate my fluid intake and output for the hour, chart quickly, ask them if they need anything, and leave to do my assessment on the other patient…as I had a job to do, a paycheck to earn, and bills to pay. I suppose this sounds much more heartless than I actually was and my motivations for becoming a nurse were certainly not based on these things, but despite it being a bit on the extreme, it provides a clear-cut example of just how much I have changed. I imagine I was so numb at times and I never took time to actually attempt to connect with the families personally as I have here at Duke. We have lived with some of these families for a while in very close quarters, sharing the same family lounge amenities, sitting around talking about our children and their diseases, sharing our lives. They became good friends, like extended family, and strangely so, even if it was a PBMTU child that I didn’t know that went into the PICU, it didn’t matter…it was as if I already knew that our families had something in common and automatically I could relate to them, just by hearing about them. We had a common connection of just being a PBMTU family. I suppose another reason is that I had not yet been ‘blessed’ with my own PICU experience and sitting on the other side of the crib, the parent who was now weeping over her own child’s critical status and having others now treating me in a similar matter. I must say that we did have a lot of caring nurses, but not all, and it only took a few to teach me of how I did not want to be professionally when I returned to work.
Ahhh, return to work, …but how could I after seeing my own baby intubated, connected to life support, multiple drips hung and infusing, vital signs unstable at times, and death standing in the shadows waiting fervently to snatch him from me. I would certainly be granted clemency if I choose to take the path of not returning, and I guess it is too early to presume that I would be able to handle going back to work and seeing critically ill children resembling Noah, possibly risking having emotional breakdowns from the bad memories. However, as emotional as I may be, I have become so much incredibly stronger. My outlook on ‘life’ has changed so much and is quite different now. I now see things in a completely different light. The compassion that I claimed before to have as a nurse, has now quadrupled. As I do certainly plan to return to work when we arrive home, I will be a much more grounded person, and a much better nurse to my patients and families. I will now have the personal experience to bring to the crib side, which will enable me to make special connections with families; and I don’t believe I have ever been able to accomplish this before. Wow, what an advantage, but it was hell on earth getting the education! God works in mysterious ways doesn’t he? When we first began this journey, I would have given anything to be that person who was just trying to keep up with the Jones’, but I see now how foolish I was to long for this. I have been blessed well beyond what I deserve. Despite how things appear on the outside of ‘our world’ looking in, we have been so very blessed to know each and every family that God has placed in our lives here at Duke and via the internet; and the ‘life’ lessons that I have learned are truly invaluable.
Just knowing where we were two months ago with Noah makes me appreciate today even more. Noah is much better now, and we are so very, very blessed for this precious miracle. But it changes nothing tonight. I remain deeply saddened, for a family dear to us is grieving the death of their sweet boy. Ignacio loved Hawaii, and although he had never been, he had really wanted to go there on a vacation. Since he was sick for such a long time, his parents were not able to take him. He even chose to decorate his door in a similar manner as Noah’s, because he saw the word ‘Aloha’ on Noah’s door and knew that this word was Hawaiian. It means love, hello, and goodbye. Tonight dear sweet Ignacio is in a much sweeter, much more beautiful paradise than where our home lies…and while we remain behind, attempting to honor his memory and never forget, we bid him a sweet Aloha. Aloha Ignacio! You will be missed! And to his family, we are praying that God might grant them peace and strength to endure this incredibly difficult time in their lives.

www.caringbridge.org/nc/ignacio

Saturday, July 26, 2003 8:31 PM CDT

Entry 140—July 26, 03/ Post Transplant Day 157/ Post Transplant Boost Day 94

What a few long, crazy days it has been for us. Noah had infusions on Tuesday, but for some reason by Thursday he needed more. Thursday's clinic day started out even worse. His central IV lines would not draw. He has two ports out of one actual IV line, and they both were not working properly in order to draw his morning blood work. We usually draw these labs ourselves, then dad takes them in and drops them off first thing in the morning. He returns to help me finish up Noah’s morning slew of medications and treatments, and then we load up the car with all of Noah’s attachments and equipment and head to clinic. By us drawing the labs ourselves early in the morning before clinic, then the results usually return by the time we arrive, allowing the doctors/ nurse practitioners to already know what blood products or other infusions that Noah may need that day…’supposedly’ to save time. Well, since his lines would not draw, we had to wait for the nurses there at clinic to do it, and we waited a long while. No luck, so they put what is called TPa in the line, it is used to break up blood and fibrin clots that form at the tips of the catheters obstructing blood from being able to be drawn out. After waiting 1-½ hours for this to sit in his lines, they returned to draw it back out and attempted to draw labs. One port was successful, the other remained stubborn and was not drawing. However, all we needed was enough for a blood lab and we were able to get what we needed. So, after nearly 4 hours of sitting and waiting, we finally were sent back to the ‘Rainbow Day Hospital’ in the clinic where all of the transplanted kids who are immunocompromised go to get infusions, and Noah received his needed infusions. Whew! What a day! Well, yesterday was a ‘Day of Rest’ from clinic, but today was a clinic day. Once again, Noah’s lines would not draw and we headed in early to clinic, second ones to arrive and thinking we would be in and out. We figured that since he had received so many infusions on Tuesday and Thursday, that we would get out of there quickly. Wrong!!!! The nurses could not get his lines to draw either. After two hours of waiting around for different ones to try, they once again placed TPa into his lines for a two hour wait, before once again spending three hours to try again only to fail, and decide what to do next. By seven hours into our visit, they decided to try the TPa again and left it in for another two hours! With a lot of turning, moving, shaking and hanging Noah inverted, we finally got just over 1 cc of blood from only one of the lines for his lab work. Ugh!!! How frustrating!!! We were there for over 10 hours just for lab draws!!!!!!! Of course, Noah’s hemoglobin, platelets, albumin and electrolytes were fine and needed no infusions. We are supposed to draw labs again in the morning, and we are praying that his lines work properly. If they continue in this manner, then he will have to be stuck to get his lab work done and would risk having to go to surgery to have his central IV lines removed and replaced. This is a huge risk since he has a shunt that runs right down into his heart and messing with his central lines places this shunt at risk for infection. As well, he would have to be intubated and anesthetized for this procedure, which scares us a bit since his latest PICU visit and our decision to take him off of the ventilator. We pray that they start working well so we can avoid this issue. Overall, Noah is about the same…no significant changes, which is good…but we would truly love to see him look at us, move around more and be able to cough, gag, suck and swallow for airway protection. We heard him give a small weak cough last night and one this morning, and we were so excited. Praise God for these wonderful things! Thank you for the continued support and prayer, and humbly ask that you would continue to keep him in your prayers.
I ran into a fellow PBMTU parent in the Duke Atrium today, and learned that their son, Ignacio, is not doing well at all and is in the PICU. I did not ask details and have not yet been to their web page to read any updates. Please, please, please pray for this young man and his family. They are from Barcelona, Spain and extremely sweet people. I could see that all too familiar expression on his face, his made my heart ache and my stomach curl…I know that look, I remember what it felt to have total despair and utter disbelief about the events taking place in front of me, and it is something that I am not sure if even Greg could put into words and offer it correct justice. I heard little hope in his voice…and it is hard to find when it seems your world is coming to an end. I pray for hope, strength, faith for this family…and I ask you to do the same. God is Great, and as Noah lives and breathes today on his own without oxygen while sitting in our apartment, I know that He performs miracles. I pray for a miracle for this family right now. We have been blessed with such wonderful friends, family and ‘web page’ supporters & prayer warriors during this journey, and we ask that you might share your love, prayers and support with this family too. Their web page is
www.caringbridge.org/nc/ignacio
We ask that you will continue to remember the other PBMTU kids, outpatient kids, and the PBMTU kids that are in the PICU, our Krabbe kids/families, and those who have lost their children. God bless and thank you so very much.

Thursday, July 24, 2003 0:05 AM CDT

Entry 139—July 23, 03/ Post Transplant Day 154/ Post Transplant Boost Day 91

Since our last update on Sunday, Noah’s meds were changed a bit. His steroids that had been weaned were increased back up to where they were, he had a chest x-ray showing reactive airway issues, so he was also started on an inhaled steroid to be given with his other breathing treatments. He was started on an antibiotic just in case there were to be something hiding, and his medication treating his CMV was increased a bit, only to be decreased again since having a new lab test return showing that the CMV DNA was back down to a less threatening level. This was done because this medication suppresses his body’s ability to make platelets and that is one of the major things that is holding us here. Since increasing the steroids and beginning the new breathing treatments, he is now off of the oxygen since yesterday. He continues to have a lot of thick mucous that is too far down in his airway for us to be able to suction out, but he has been able to tolerate having in his airway thus far. We continue to desperately pray that it won’t block his airway at anytime. He has had on very rare occasions given a very weak cough or gag, but it is not yet enough to do the job of clearing his airway on his own. We pray that God continues to heal his reflexes and his ability to keep his own airway open. Yesterday’s clinic day was long, about 9 hours, but he received quite a bit of blood products and infusions, so hopefully tomorrow will be a breeze for us. He continues to still have some withdrawal symptoms…jerking/shaking, agitation, crying…but remains asleep almost all of the time. We are very grateful and praise God for his being able to remain at ‘home’ with us, that his airway has remained open and he is now on room air. We are so very grateful for all of the prayers for him this week. We have been so very exhausted, but did get some good rest last night. We can only pray that things will get a little easier for us from here on out. We know that with Noah, things will never truly be easy, but we will certainly pray and hope for things to get easier than they are now. We continue to pray for little Manny. Please visit his page and offer him and his family support at
www.caringbridge.org/sc/mannymiramontes
There are a lot of kids that need prayer, so many that I know that I cannot mention them all. There are a lot of parents that need prayer too, who like us, are spending every waking moment and every last ounce of time and energy to keep their child alive and comfortable and is so desperately weary and needs rest. We pray for them, too. We want to wish Andrew and his family the best as they make the trip home again!!!!!! Congratulations!!!!! Please wish them well at
www.caringbridge.org/page/andrewshope
Thank you to all of you who have visited Manny’s site this past week and offered your support. I know they truly appreciate it! God bless you!

Saturday, July 19, 2003 8:17 PM CDT

Entry 138—July 19, 03/ Post Transplant Day 150/ Post Transplant Boost Day 87

Noah’s labs showed today that his Albumin was back within the normal range, as well as his platelets and hemoglobin, so he needed no infusions today in clinic, and we were in and out in just 1 hour!!! I believe that is a record! Wouldn’t it be nice if each clinic day were like that? So we had time to sleep this afternoon…wow, how refreshing and definitely much needed! His methadone and steroids were weaned a lot this past Tuesday and within a couple of weeks we hope that he will be off of these medications. His CMV came up positive again, showing CMV DNA floating around in his blood system. If this were to become a full-blown infection, it could be extremely dangerous for him. He has been getting an IV medication but only Monday thru Friday, so the frequency was increased today for this medication in hopes of putting this issue at bay. Noah is still requiring a lot of suctioning, and yesterday he required oxygen to keep his oxygen saturations up into the normal range…he had been in the low to mid 80s We are praying that this improves. Apart from these issues, he seems to be doing all right. We are now trying to recover from this week, and since we are keeping extremely busy, we have not spent much of any time on the computer…thus, we have not made very many updates. Hopefully, we can do a little better about doing this to keep everyone updated.
We would like to wish Jeremiah, a fellow PBMTU kid transplanted for ALD (another leukodystrophy similar to Krabbe), a huge congratulations for being able to make his journey back home!!! We wish him the best and will keep him in our prayers. www.caringbridge.org/ia/jeremiah
We humbly ask that you would continue to remember Noah in your prayers, and all of the other PBMTU kids (inpatient, outpatient and PICU). Noah will be having another donor cell test drawn on Tuesday, and then we should hear back by the end of next week as to how his donor cells are doing. We are praying that they are still growing like crazy! Thank you for your continued prayers and support, and for checking in on Noah and writing in the guestbook despite the lack of updated journals. God bless.

Just a quick thing I wanted to add on. There is a young boy and his family that need your prayers desperately!!! Little Manny, who was transplanted for ALD (another leukodystrophy similar to Krabbe called Adrenoleukodystrophy...ever heard of Lorenzo's oil???) and was in the PICU with Noah for a short time. He is having some trouble right now trying to recover from the problem that placed him into the PICU a couple of months ago, and his family has been given some devastating news about Manny's loss of sight and possible diminished hearing. We can relate some to this terrible news, as Noah's eyes still remain dysconjugate (not together, each eye looking in different directions) and rolled up or to the side. It is not certain that he can see. Thus, we recognize how devastating this news is. You all have been so wonderful about visiting other kids web pages when I list them and offering your support. We once again ask you to visit another site, Manny's site, and offer him and his parents some much needed supports, letting them know that you are saying some extra prayers for him. We are so very grateful. Thank you.
www.caringbridge.org/sc/mannymiramontes

Sunday, July 20, 2003/ 23:30
Just wanted to add on a quick note about the last 24 hours that we have had. Last night Noah continued to sound terrible, his lungs crackling and wheezing full of mucous and fluid. He needed oxygen and suctioning all night. He began having withdrawals from the Methadone wean done on Tuesday, jerking all around, crying, having a heartrate up to the 180s and breathing really irradically. It was very scarey for us, very scarey and stressful. We had to sleep in the living room, dad in the recliner and myself on the sofa, while Noah had to sleep in his bouncer seat near the huge oxygen tank since the nasal cannula tubing is only about 6 feet long. We will have to get a longer one that will reach to the bedroom. Anyhow, I was able to sleep a few hours, but dad was worried so much that even the pure exhaustion that we have was not enough to fight off the worry and allow him to sleep...he sat in the recliner, studying the monitor for a few hours until early morning when we got up to give Noah's early morning medications and treatments. He still sounded very, very bad...we thought for sure that if he did not start to improve, that our stay in the outpatient world would very soon be coming to a close. We had been desperately doing chest PT, turning him frequently, suctioning him every 15-30 minutes, giving his respiratory treatments, all in hopes of sparing his airway. It is so difficult to explain the terror involved in personally trying to save your child's life, for fear that he may die if you aren't able to suction out enough mucous so that he can move air in and out of his lungs. That if you happen to stay asleep too long and not wake up in time to suction him, that he may develop a mucous plug, which will plug part of his lung, causing it to collapse, and putting him respiratory distress, placing him near death's door. Noah had this happen before in the PICU, and it is so very scarey, because we are in our livingroom, away from doctors, nurses, respiratory therapists, etc. It has been such a hard week, as this has been going on pretty much since Wednesday night of last week. We emailed Dr. K and told that we should go to the clinic, have a chest x-ray done, and when packing the bags, we both thought that we would be packing to stay, because Noah would be admitted to the unit. Well his x-ray showed a little collapse in the upper right lung, and some constriction in his airway (similar to something like asthma). His steroids were increased a bit and he was started back on another antibiotic. We are supposed to go into clinic tomorrow too, to have a respiratory viral battery done to test for possible infection. We are praying that Noah is simply having some sort of systemic allergic type response and that the new steroid dose will help him out. Please continue to pray that Noah's airway is able to stay open and will clear of the mucous that is in his lungs, that his collapsed areas will open up and fill with air. We pray that God will help us to figure out what it is that is causing Noah to retain fluid, making his albumin drop, and preventing his lungs from staying clear. Thank you for your much needed prayers, and especially for praying for the other kids, in particular Manny and his family. God bless.

Friday, July 18, 2003 0:42 AM CDT

Entry 137—July 18, 03/ Post Transplant Day 149/ Post Transplant Boost Day 86

We are exhausted. Yesterday Noah began to sound a little junky. This is usually an indicator that he needs suction and does not cause us much alarm. The norm lately is a quick suction and he clears right up and stays that way for several hours. This was not to be yesterday. Try as we might we couldn’t get him to clear and if we did it lasted for only about 30 minutes. Buy midnight we were a mess trying to keep his airway clear. It is terrifying for us to not be able to clear him, we worry so much that he will go into respiratory distress. So all-night we stayed up and as a team worked to keep him clear, enabling him to maintain his sats in the low 90% range. At 0300 we wrote Dr. Kurtzberg to inform her of his situation to prep them for when we came into clinic. The day prior they had dropped one of his breathing treatments and one of his diuretics doses which helps him to clear fluids, so we gave those deleted treatments at about 0330 figuring at this point we had nothing to lose. It still did not seem to help. We suggested to Dr. K that his albumin might be low; in the past it has caused his lungs to get very wet. When we came in to clinic they informed us it was low and they had a IV ready to go for him already. We were glad we gave her a heads up. Noah still sounds wet and is requiring a lot of work but we are able to clear him and even were able to nap for about an hour today. We are sorry we have not updated for several days, but as you can tell we have been very busy. We appreciate all of your continued support and prayers. Please do not worry too much after reading this entry. We will get it under control, and do whatever it takes to keep his lungs clear until his albumin gets back to were it should be. It is nice to know for a change what is causing the problem and how to correct it.

Sunday, July 13, 2003 0:43 AM CDT

Entry 136—July 12, 03/ Post Transplant Day 143/ Post Transplant Boost Day 80

We wanted to first begin by once again offering our sincerest condolences for the Nicoll family for the loss of Douglas. As well, we were informed this morning that little baby John Evan had passed away in the PICU on Monday. We are so desperately sorry for this family. John Evan, the only child of a very young, sweet couple, who had a very rare, undiagnosed and fatal Metabolic disease, born just over a couple of months ago. There is no web page that we can provide, so we must offer our support in prayers, asking God to be with this family during this extremely painful time. We got well acquainted with this family during Noah’s recent stay in the PICU, and have been praying for healing for their son. It is so hard to understand God’s plan, when he spares the life of one child, and takes another…when one lives a ‘normal’ healthy life, and another is stricken with a terrible fatal disease…why any child must suffer…what are we supposed to learn from all of this? Some of the obvious lessons are that ‘life is so very short’, ‘there are no guarantees’, ‘God is in control no matter what we as humans try to do to alter the situation’, ‘never take anything…ANYTHING, for granted’, ‘savor the moment, because at any second it could all change…and you just may not like the changes that unfold’, and ‘pray…pray, pray and pray some more’. We are ‘high on life’ right now, so unbelievably happy that Noah is alive and with us outpatient and making small improvements with each day…but we live each day knowing that at any moment our worst fear could happen…Noah would be taken from us. We are so very well aware of how fast things can go downhill, but we also know that we must value the good times. So here we are…totally ecstatic that Noah is finally on a path to healing. PRAISE GOD!!!!! We don’t understand why he has spared Noah’s life thus far, nor do we know the plans he holds for Noah’s future, but we are certainly giving him all of the praise for these wonderful blessings. We are so grateful for the tremendous strength, courage and tenacity that God has blessed Noah with. We are amazed each day by his improvements. For those of you who scan the guest book (which is totally fine), you may have read the entry left by a fellow prayer warrior and well-wisher, Jackie Teichmann from Huntington Beach, CA. She wrote a quote that was special to her during a difficult trial in her family and we were truly touched by it, as it characterizes Noah’s fortitude throughout his short life. It reads, “THERE IS NO CHANCE, NO DESTINY, NO FATE THAT CAN CIRCUMVENT OR HINDER OR CONTROL THE FIRM RESOLVE OF A DETERMINED SOUL.” Thank you Jackie T. for sharing this remarkable quote with us all.
As for an update on Noah’s status, today in clinic Noah received some more potassium, as the diuretics he is on cause him to lose a lot of his potassium and he requires supplements to replace his loss. As well, last night Noah broke out in a bad rash and began wheezing a bit after his midnight dose of an antibiotic. We gave him some Benadryl and an extra breathing treatment to get him through the night until clinic this morning. He did fine and we skipped his morning dose until we could get him into clinic, but he still had the rash today. They want us back tomorrow to draw some blood cultures just to make sure that nothing is growing and that he no longer has a need for any antibiotics. We were supposed to be off tomorrow and Monday, but we want to make sure that Noah is out of the woods completely and not play with fire by taking chances. Hopefully it will be a short visit. However, ironically, the rash could be from graft vs. host, meaning that Noah could have some donor cells growing. There is a lesser chance of this than an allergic reaction, but who knows…we certainly have learned that anything is possible!! We continue to pray for his donor cells to grow, his enzyme level to rise, and his brain to continue to remyelinate. We took time today to stop up onto the bone marrow floor to visit with some of Noah’s inpatient nurses and some of the other families that remain inpatient. Degan (Krabbe kid) looks great and appears to be doing well. Ignacio needs a lot of prayers right now after recently being in the PICU. We continue to pray for Daryn and Manny as well, who are trying to make their way to outpatient. Elijah and his sister Alissa (Krabbe kids) need plenty of prayer right now. www.caringbridge.org/il/elijahbarron Please remember our buddy Jordan, Nolan, and so many, many other great kids in outpatient that are working so hard on trying to getting home. Please continue to remember those families/parents who continue to grieve the loss of their child/children, and our non-transplanted Krabbe kids. There are so very many kids that need prayer, and we would not want to forget any of them. We are so grateful to all of you who diligently keep all of the kids and families in your prayers, as we know that it means so very much. Thank you. God bless.

Friday, July 11, 2003 8:14 PM CDT

Entry 135—July 11, 03/ Post Transplant Day 142/ Post Transplant Boost Day 79

We have had a pretty decent week, again. Seems so hard to believe that we have gone more than a week without a catastrophe, but definitely easy to enjoy. Noah has started bolus feeds three times throughout the daytime and then has continuous feeds at night. He has been doing really well on the bolus feeds, so we may be able to increase them this next week and gradually reduce the need for continuous feeds. He needed some blood products yesterday, but for some reason things were extra hectic in the clinic and they couldn’t seem to get things in sync, and so our day was so very, very long. It was one of those clinic days that we had heard about from other families discharged to outpatient before Noah; days that seem to last forever and are extremely exhausting. Well, we ended up having to carry over our clinic visit to the inpatient treatment room since Noah’s blood was just beginning to infuse and the outpatient clinic was closing down for the day. We did not get home until 11 pm, just 13 hours after arriving at clinic at 10 am! This was just in time to give all of his midnight medications and breathing treatment. What a very long day.

Well, we already reported that Noah’s donor cells were up to 16.5rom 14.5but we just recently learned about his enzyme level. He is up to 0.6 from 0.5. This is on the lower side, but it is at least some enzyme that will surely help break down the toxins, which were damaging his myelin; therefore, he will be able to myelinate some more. We are so delighted, and continuing to pray that these donor cells keep growing. They probably won’t check another donor cell test until Noah is about 6 months out of transplant. However, given his past of growing cells and then losing them, they may keep a closer eye on them. We continue to pray for his complete healing, realizing that he is certainly on his way! We continue to say THANK YOU to all of you praying for our sweet baby Noah. God is certainly blessing him with some miracles, and we are so very thankful! Please continue to remember all of the other PBMTU kids, those in outpatient, in PICU (baby John Evan), and our Krabbe kids, and for a family who just lost their son named Douglas who passed away in the PICU. We pray that God will wrap his loving arms around them and bring them peace during this very tragic time. Please visit their web page at www.caringbridge.org/co/nicollbrothers We never met this family, as they were already outpatient before Noah began the transplant process, but both of their children were transplanted for a Metabolic disease. Please offer this family support. Thanks again and God bless.

Tuesday, July 8, 2003 5:10 PM CDT

Entry 134—July 8, 03/ Post Transplant Day 139/ Post Transplant Boost Day 76

Well, as most of you who have been checking up on Noah have found out, we have taken off a few days from writing any journals to just catch our breath, get some rest, adjust to doing ALL of Noah’s busy care ourselves and spend time together as a family for a change; therefore we have not gotten on-line to update his web page. Noah has been doing quite well, still requiring no oxygen to keep his oxygen saturations in a normal range. He has needed quite a bit of suctioning still, but we are managing it all right for now. He still sleeps most of the time, not giving eye contact when he does crack his eyes open, but we are delighted to just have him home. We have only had to go to clinic every other day so far, and today we were told we only need to go 3 times a week for now (Tues, Thurs, Sat), until further notice…the goal being only once a week until we get the green light to go home. A few of his meds have been changed a bit, taking away one of his blood pressure meds, now only on two. His steroids and methadone (for withdrawals) were both decreased a bit. We will begin small bolus feeds tomorrow. We pray that he will tolerate this well. We hope to get Noah measured for a ‘Kid Cart’…sort of a cross between a stroller and wheel chair, which will offer him the proper support he needs since his disease has left him unable to really move at this time. We cannot say enough how truly grateful we are for all of the prayers and support. God has truly blessed us, and we are forever grateful. Noah remains still quite sick and devastated from his disease, but he is alive and at ‘home’ with us…we are so very happy. We ask that you would continue to remember Noah and his need for healing, the other kids that remain inpatient on the PBMTU (Manny and Ignacio who we are told need some extra prayers right now), Krabbe kids (Degan and Daryn who are now on the bone marrow unit being transplanted), all of the kids/families in outpatient clinic (Jordan…who was really sick last night), Alissa (Elijah’s sister) who needs some extra prayers. We know there are so many more prayer requests that we have forgotten to mention. Jillian and Jacob need a lot of prayers right now too. Thank you again…Kelly, dinner was fantastic and we are forever grateful to you for the tremendous kindness you continually show our family! THANK YOU!!!! Thank you mom and Natalie for coming to visit and helping us get settled into the apartment. God bless.

Friday, July 4, 2003 10:21 PM CDT

Entry 133—July 4, 03/ Post Transplant Day 135/ Post Transplant Boost Day 72

Well, we are now in our apartment, not yet settled, but making every effort to get there. Yesterday was long and tiring, after getting discharged a little behind schedule…around 3:00 pm, we went directly to the apartment to meet the various home health people who were to come and deliver equipment, set it up and meet the home health nurse to discuss medication schedules. Unfortunately there was no discharge nurse to help us out with all of this because the person who was doing this job had just quit, so the PBMTU staff was trying to do their best to get us set up for success, but it went a little rough. Well, all of the home health people were late and so all 20-something of Noah’s medications ended up being 2-4 hours late, which got us off to a bad start. We had a rough night trying to get them all back onto schedule by morning. Noah missed one of his doses of his medication that helps dry out some of his mucous…so he ended up having a TON of mucous building up in his airway, scaring us a bit as he needed so much suction and his lungs sounded so VERY bad. He made it through with our constant suctioning and has improved throughout the day. We had our first morning of lab draws and Dad drove them in to the clinic. Then once his morning meds were started, we loaded up the car and went to the outpatient clinic for our first day. We figured Noah would need platelets since they were low when we discharged, and sure enough that is what he got when we arrived. We were there just over 3 hours, then able to return home to nap for a while and finish a bit more unpacking. We will not be going to clinic tomorrow…we have the day off. YEA!!! So far, so good. Noah seems to be pretty comfortable, but remains drowsy and sleeps quite a bit. His eyes are still rolling around and not focusing…still praying that this corrects itself and his vision is okay. We are so pleased that he is doing this well…after being told the very worst, not sure if he would even make it out of the hospital. His donor cell test returned at 16.5%!!! Up 2%!!! YEA!! Praise God!!!! It is the tiniest bit, but it is at least moving in the right direction. We are praying so hard that the donor cells he has is sparing his brain from further injury, and that he continues to grow donor cells! GROW DONOR CELLS GROW!!! We continue to pray for the kids inpatient and in clinic. Please remember Manny, who is trying to recover on PBMTU since being very sick in PICU a few weeks. Thanks again for all of the wonderful support and prayers. HAPPY INDEPENDENCE DAY!!! Noah is celebrating his freedom from being in the hospital, and we couldn’t be more thrilled! God bless!

Friday, July 4, 2003 6:34 PM CDT

Entry 132—July 3,03/ Post Transplant Day 133 Post Transplant Boost Day 70

Today is Debbie’s Birthday and I had made some covert plans for the celebration. Nothing to extravagant, just a good dinner in a nice quiet restraint. I wanted to give her a chance to relax and unwind for an hour or two after a long and stressful month. I started my planning a week out, and Kelly from Hunters Hope was kind enough to volunteer her time to stay with Noah. All the plans were laid so I could surprise Debbie with a secret dinner date. All the scheming is cancelled now because of a very welcome but unexpected dinner guest. We will not be dinning out tonight; Kelly’s services will not be needed. Noah decided to upstage Dad’s surprise with one of his own… and he will be joining us tonight for dinner. For the first time in almost six months we will be together as a family outside of the hospital. Noah has given us both an incredible present. He has done so well that tomorrow he will receive the coveted title few thought he would ever see, the title of outpatient. We will load him up in the car seat and take him back to the apartment. I will go get some take out somewhere, set the table, light a candle, and we will celebrate Debbie’s Birthday with Noah in attendance. It will probably seem unreal at first, it has been so long, but Noah has earned this trip and we are excited to have him outside of the hospital. Birthdays in or family have always been a celebration for everyone, a chance to celebrate the birth of someone we love, and to thank God for giving him or her to us. Thanks Noah for make Mom’s birthday a special one, that even though it will be simple and quiet it will always be remembered.

Wednesday, July 2, 2003 7:33 PM CDT

Entry 131—July 1, 03/ Post Transplant Day 132/ Post Transplant Boost Day 69

This entry will be short…but filled with nothing negative! Although we are still a bit worried about his blood culture, Dr. Martin doesn’t think that there is really any need to be real concerned as Noah has not exhibited any signs or symptoms of infection…no fevers, no alterations in his blood pressure or heart rate. Good, good, good!!!!! So, we are continuing to pray that this will amount to nothing more than just something to keep us on our toes…but we need little reminding of how quickly things turnaround for the worst. For now, we are focusing on his discharge plans…possibly in 2 to 3 days!! It is all so surreal, after 132 days of struggling very hard to get to this point…we are finding it hard to believe. Noah’s blood pressure has been stable…Praise God! Although he is still having quite a bit of mucous, needing quite a bit of suctioning, he has been able to maintain his airway, only needing a little bit of blow by oxygen and keeping his oxygen saturations at 96-99 Praise God!!! He is tolerating his tube feedings, still not quite ready for bolus feeding and will remain on continuous feedings for a few more weeks, but we are not complaining, because he is tolerating them well…and for those of you who have been following Noah’s progress for the last few months you know that he has had a tough time getting up to tolerating 30cc/hour! Praise God!!!! We want to say Thank you to all of Noah’s web page readers, supporters, and prayer warriors, who have been an essential asset to us throughout this entire journey. Thank you. Noah’s best buddy Heidi is here to watch Noah while mom and dad go find an apartment for us to stay in during the outpatient phase. We are excited by Noah’s progress and will let you know how discharge goes!

2 July 03---Midnight
Just wanted to add a little something to this journal...a journal that was written over a day ago. We are now in an apartment...MUCH nicer than the hotel and will be great for us when Noah is outpatient. It is nearby some of our good bone marrow friends/family...Jordan, Noah is coming home, buddy!!!! Noah is gonna be discharged in about 12-14 hours!!!!!!!! Somehow we have to try and sleep tonight with all of this incredible excitement!! We are literally busting at the seams, as Noah is sleeping through it all, not aware of the magnitude of the situation. We hope to make our confetti gauntlet marathon escape tomorrow around 1:00 or so in the afternoon. We will certainly take plenty of pictures and post them. We are excited and nervous, scared that Noah is always at risk of having to come back in at any point if he were to get sick again, delighted that this dream has finally become a reality. We will be in doing the whole daily clinic routine, but we have not yet experienced it for ourselves and will surely have more things to write about this part of our transplant journey. The sooner Noah makes his own platelets and is stable, then we are off home to Hawaii. Basically this is one of the only things keeping us here. We will find out about his donor cells either tomorrow or Friday in clinic. We are praying they are higher. So far, no more positive blood cultures and we are so relieved. He is being covered with antibiotics. We will be leaving behind a lot of good friends...not just other families and kids, but the nurses have become a part of our family and we will miss seeing them each day. Thank you to each of them for all of the great support and care...and laughs (Keri Garrison). We will visit...only as a visitor. Thank you to everyone for all of the great prayers and support. It is so nice to be able to share good news. We owe all praise and glory to God. Noah is our miracle baby. Praise God.

Monday, June 30, 2003 10:57 PM CDT

Entry 130—June 30, 03/ Post Transplant Day 131/ Post Transplant Boost Day 68

Well, we begin this week with a little excitement, the news that Noah ‘MAY BE’ discharged to outpatient status this week…MAYBE! But ‘maybe’ is certainly encouraging news. There are a few issues that must be resolved, in particular, Noah must be able to sustain low enough blood pressures on his G-Tube medications, successfully weaning off of his IV hypertension medication. They have turned this IV medication off today, and so far his blood pressures have been within an acceptable range. For the most part he is in a position where his care could be managed in the outpatient setting. We were told that he might even be discharged as early as Thursday, July 3rd, but again, we will have to take things a day at a time. He had his donor cell blood work drawn this morning, and we will receive the results of this test by Thursday or Friday of this week. We are praying that his donor cells are still growing and thus we will see an increase from his previous 14.5% donor cells from 2 weeks ago. Despite how well he is doing, we have received some alarming news this evening…again…bad news to follow the good? He has tested positive for a blood culture in one of his central chest IV lines (in other words, his lab work shows that he has bacteria in his blood stream). Ordinarily we would be alarmed, concerned, but not totally freaked out about it…because they would treat him with antibiotics and since he has not been running any fevers, then he would probably be all right. HOWEVER, because his brain shunt goes from the middle of his brain down into the main vein leading into the heart, this means that this shunt is now at high risk of having bacteria to start growing in it and eventually getting up to his brain and into his spinal fluid…VERY, VERY, VERY DANGEROUS!!!!! We are very worried, although we were told that this one positive blood culture could possibly be a ‘false positive’ and there was a lab error or contamination. They redrew the labs this evening and started him on an antibiotic to cover him until tomorrow when the attending bone marrow doc is back. There is only a fellow doc here during the nights. If he does indeed have a positive blood culture and his shunt is threatened with infection, then they might have to take him back to surgery and put in another external drain, …which means we would be back to where we were before…and definitely not going to be close to discharge. We are just praying and continuing to place him in God’s hands. We have no other choice. Thank you for all of the prayers. Again we are faced with another bump in Noah’s road, just when he was starting to move slightly forward.
Please continue to remember him in your prayers, as well as little John Evan in the PICU, Manny who is recovering on PBMTU after returning from the PICU just days ago, the other kids on the PBMTU and in outpatient, and the Krabbe kids, too. Please remember those families who continue to grieve the loss of their children, most recently Marianna and Savvas Koumpouras. Again, I am asking our family members and friends, strangers, acquaintances to visit their web page to offer them support and encouragement. Thank you to those of you who have already done so and to those who are going to do so. Just knowing that there are people praying and thinking about you means so much. Thank you so much. God bless.

www.caringbridge.org/nc/koumpourasboys

Sunday, June 29, 2003 4:46 PM CDT

Entry 130—June 29, 03/ Post Transplant Day 130/ Post Transplant Boost Day 67

There are times while we are children growing up that certain experiences become ingrained in our memories. Sometimes it is because of a painful or positive occurrence, or maybe we realize the gravity of the moment and file it away in our memory banks for future reference. For most of us our childhood memories are treasures to be pulled out and viewed when the times are tuff, or used as an escape when we need it most. I have a few of these memories tucked away, like the Apollo moon landing, our nations bicentennial, my first trip to Disneyland, camping with family, several great Christmas’s, Cub Scout events, and the list goes on and on. Every one of them is important in it’s own way and uniquely mine to remember and share with loved ones. So this brings me to the heart of the matter. As I was sitting in Noah’s room late at night, I thought what would I want to share with him right now. I could never share a similar experience of when I was sick and in the hospital, because fortunately I never have been but to visit or to be with others. So what could I share with him? As I gazed out his window one of those childhood memories came flooding back to me and I realized I could share with him the memory of one night. I was taken back to my scouting days, when we hiked an entire day out to a small lake, secluded in the woods of western Washington. Because of the distance we had to hike, we spent the night on the banks of this calm little lake, and would leave the next morning. I remember that time as almost mystical and magical, for after the campfire talk was over and the fire was put out for the night, it became silent and still, all of us to tired from the hike to offer much resistance. I laid in my sleeping bag staring up into a crystal clear black sky, the sting of cool air blowing off the lake on my nose. It was the best night sky I’d ever seen, so good I had to remember it for all its wonder and majesty. I lay there for hours taking it all in and pondering the magnitude of what I was seeing. Everything was visible from the haziness of the Milky Way to the rare falling star, unfolding on a pitch-black canvass, speckled with billions of spots of light. I felt privileged to be able to witness this display of God’s power in all its beauty. As this memory came back to me a realized how important it will be to share this experience with Noah. He may not be able to hike great distances, but he can lay with his Dad on a blanket in the back yard, and wonder as I did so long ago. And on a clear night gaze at the heavens and hear the story of when his father was young, and share my memory, hopefully making it his own to keep. Because after all, aren’t our dreams, hopes, and experiences the best thing we can leave to our children sometimes?

Today Noah was pretty much unchanged; he is still getting rid of a lot of junk left over from intubation. Debbie and I spent all day and night giving him chest PT and suctioning him. He was sating in the low 90’s and we were frustrated and unable to figure out why. Until we looked at his oxygen settings, and realized it was turned off. We don’t know when or who did it, but once we gave him just a little bit of O2 he shot back up to the high 90’s and was a lot more comfortable. We have been holding him a lot trying to make up for lost time. This morning he was doing great sating in the mid to high 90’s with no O2 support. We will take it day by day. He is beautiful as ever and everyday he moves forward we get closer to taking him home. Please continue to keep all the families and children of the fifth floor in your prayers as well as those who suffer else where from sickness and pain. Peace….

Thursday, June 26, 2003 10:29 PM CDT

Entry 129—June 27, 03/ Post Transplant Day 128/ Post Transplant Boost Day 65

Death contently lurks the floor of the bone marrow unit like a shadow. It peaks in through the blinds and nocks on doors, sometimes in the dead of night or the light of day. It continually calls to our children. We have seen it’s face many times, dark and featureless, cold and terrifying. You come to know it, and it is ugly. When you are forced to stare it in the eye it saps your strength, it is unknown and horrifying. It hovers over your child like a black storm cloud, rolling, large and awesome, sometimes blotting out the sun. When death comes calling on your child you are helpless. You can only surround them with love, and scream in your sorrow to be left alone. You can see it’s icy hands touching your baby but you can’t wave them off. It wont barter with you no matter how much you try, it constantly lurks in the cold dark shadows of the room. You can’t win against death, it wears you down with patience, and one day it will inevitably win. Your only hope is that your child has enough strength to live for another day, to fend off deaths call from the shadows. What sustains you when death comes for your child? Faith…Faith carries you because you know that death is only a phase of life. Death will come for all of us, but for those with faith we know it is only the beginning of wonderful adventure. There has never been a doubt in our mind that if Noah was taken from us we will see him again, healed and without pain. We know he will not go unless God allows it, and so this last week we told the doctors to extubate him and we would see what God wanted. We were told that he would probably not make it, and ushered into a room on the floor to begin the vigil. Well, a week latter he is no longer on oxygen, he is doing great and for now… death has left the room. His donor cell counts and MRI shock all the doctors, he has been on his own and God took control. We are thankful that we placed his destiny in His hands. Our God is an awesome God.

Thursday, June 26, 2003 1:10 AM CDT

Entry 127—June 26, 03/ Post Transplant Day 127/ Post Transplant Boost Day 64

These are just some thoughts that have come to mind...

I have not yet made it to bed, still awake wrestling with the thoughts of why things seem to happen and turn out the way that they do. There have been so many innocent lives lost since our arrival at Duke in January for the transplant that is going to save our son’s life…Mitchell, Reese, McKenzie, Travis, Ryan, Kendal, Garrett, Jose, Juan, Aristophanis, and Hermes here at Duke, and two from our Krabbe family who were not transplanted, Makayla and Natasha. Please forgive me if I have forgotten a name, for each deserves recognition. We are only left to try and reflect upon our lives, attempting to discover our purpose in ‘life’ and why we’re chosen to remain here, why these things happen to children, why ‘our’ children; and there seems to be little accomplished with this. We don’t have the answers…no answers, only promises that we are not alone in this hell on earth…those promises that God made and are in his written Word. However, words are not always enough to comfort an aching heart. I have certainly found this to be true. We have been told that ‘God doesn’t give you more than you can handle’, ‘God has blessed you with Noah because you are strong’, etc., which can only do so much to comfort you when you can’t even see God in the picture. I have thought…the latter of the two statements is similar to saying that ‘It couldn’t have happened to two better people’…but isn’t this something that is said when referring to something ‘good’ happening. Please don’t misunderstand me, Noah is beautiful…he is the most beautiful baby…perfect in so many ways. We are able to rejoice tonight, with the turnaround that Noah has made, demonstrating the handiwork of God’s power, and we are so very, very blessed. However, just 6 doors down, a family is in great anguish after having lost their second son hours ago, just a mere month apart from losing their first. My heart cries out tonight, WHY GOD!!! HOW CAN THIS HAPPEN!!!!! I cannot begin to understand their grief. They came to Duke with similar hopes as we, that the lives of their only sons, which were threatened by fatal disease, would be spared with treatment…granting them long lives together as a family. They will return to their home with nothing…nothing more than broken hearts and spirits. I visited their web page, and like many of you have said on Noah’s page that you have never written on his guest book because you did not know what to say, I too did not know what words I could offer to comfort them. None; because as I knew by living through my own experience, words can be of little to no comfort when your heart is in complete turmoil. However, I depended upon these words to get me through when the prognosis for Noah was so very grim just weeks ago, preceded by months of complications and bad news. So I left our simple words of encouragement on their web page, unsure when they would find the energy to even read it, but hoping that they would at least know that we care. As I sit here tonight, confronting my emotions and attempting to make sense of it all, I felt compelled to write some things down, and so I have done. There are so many that need prayer tonight, as Manny and little John Evan remain in the PICU, the other PBMTU kids, Krabbe kids, outpatient kids, and parents who are now without their kids, …but I am asking, each and every person that we know, and those we don’t, who sign Noah’s guest book, to please, please, please visit Hermes & Aristophanis guest book and offer your support. To let Savvas and Marianna know that they are being lifted up in prayer during this most difficult time in their lives. The words may not bring much comfort, but they will get them through. The comfort they need must come from God, and we will pray for God’s blessings of comfort, strength and peace for them tonight. Thank you for all of your support and prayers. Your guest book entries are VERY important to us, and we truly appreciate reading them. They have helped get us through some mighty tough times and we are so very grateful to the dedication that so many have shown in leaving words of encouragement. Thank you.
We rejoice in the blessings that God has granted us, mourn for our friends who are grieving with great sorrow, and ask that God will be with us all. Thank you for your ear, God bless.

www.caringbridge.org/nc/koumpourasboys

Tuesday, June 24, 2003 9:03 PM CDT

Entry 126—June 24, 03/ Post Transplant Day 125/ Post Transplant Boost Day 62

Noah remains about the same, doing better than everyone expected, but still has a long road ahead if he is to pull through all of this. Some of his medications were changed from IV to oral medications to get us closer to discharge. He was finally weaned off of his pain medication drip, and his steroids are being gradually weaned. He is up to 1 oz./hour of tube feedings and doing fine. After a few days at this rate, they may try to start giving him small boluses, but we will have to wait and see. Noah is being held a lot now, it is hard to let him down. He still needs quite a bit of suctioning, but we are able to keep his airway clear this way. We have had a rough few days, after learning that our dog, Yzerman—a Dalmatian, passed away suddenly. We have had him since we got married and he would be 7 this year. He was a member of our family and this has been a tough blow, since he was our only ‘child’ for over 5 years. We are so very grateful for Tony, Moana, and the Mailo family for helping us out with him, since we have not been there. Thank you guys, we are so blessed to have such great neighbors. However, we also have some good news. We had first received the preliminary results of the comparison of Noah’s MRI’s from January and the one done last week. Without looking at the actual myelin, it showed quite a bit of atrophy (shrinkage) of the brain…some attributed to steroids that he has been on so long, and some to Krabbe disease progression. So when we learned of this, we were pretty certain that we would hear that his myelin study would show a breakdown in his brainstem, meaning that our decision not to reintubated him would be the best and that we should just let him go. However, today we received the portion of the report that listed info about his myelin. It seems that he has been able to make myelin on his own, meaning that the enzyme must be present for him to be able to do this, but it was much less than what it would be in a normal 9 month old without a neurological disease. BUT, this is good news. We inquired about his brain stem, and Dr. K said that she had not received anything on his brain stem, so if she would have to guess, she would assume that the doctor who reads these types of MRI’s would tell her if he saw damage in the brain stem. PRAISE GOD!!!!!!! WHAT A BLESSING!!!! We are absolutely so shocked!!! Thank you to each and every one of you has said prayers on Noah’s behalf for healing. We are truly grateful to you!!! God bless you!! They will do another donor cell test next Monday, so we are praying that his donor cells are growing, growing, growing so that we see a number higher than 14.5 We pray that Noah continues to grow stronger, his lungs clearer, and that he continues on the road to complete healing. We are so very, very grateful for the blessings God has bestowed upon us this last week. WOW, we could never have imagined Noah doing this well. God is in control, and thank goodness for this! We continue to humbly ask for your prayers for Noah’s healing, and for the other children here on the unit, in PICU and those in outpatient. We also ask for prayers for all of the docs and staff taking care of Noah.

I don't like to write about these sort of things, but I do it, because it is in times like these that people need prayer most. I am writing to report the loss of yet again, another sweet bone marrow child who has passed away in PICU just a short time ago. Hermes, brother to Aristophanes who passed away just about a month ago in PICU, has died today, after a long struggle to survive. His parents Savvas and Marianna need so much support, prayers and encouragement right now. They will be returning home to Greece childless, dreams and hopes for a brighter future for their family totally demolished. They just recently set up a "CaringBridge" web page for their boys, so we are asking that EVERYONE that visits Noah's web page, please just visit theirs too, and even if you don't know what to say...Please just let them know that you are thinking of them and will be praying for them. Their web page is www.caringbridge.org/nc/koumpourasboys

Thank you so very much, I know it will mean so much to them, and it certainly means a lot to us. God bless.

Sunday, June 22, 2003 10:28 PM CDT

Entry 125—June 22, 03/ Post Transplant Day 123/ Post Transplant Boost Day 60

Noah has done relatively well this weekend, but managed to give us a few scares each night by having a few episodes of high respiratory rates in the 50s (normal for him is in the 20s). He is back up to 1 L of oxygen and is still having some cloudiness in the same areas of his lungs. We are so pleased with how well he is holding up. He has opened his eyes quite a bit, but does not give any eye contact. It is difficult to determine if he even sees anything, and if not, then we are not certain if it is from medications built up in his system or if his disease has now made him blind. This is somewhat disheartening, but hopefully it is just medication that needs time to get out of his system. They are continuing to wean him off of the pain medications slowly to prevent withdrawals, and he might be off of them as early as tomorrow. He is still on his high blood pressure continuous drip and will have to be off of that before there will be any talk of us taking him home. As his steroids are weaned his blood pressure should come back down to normal and they should be able to wean it off. He is still a little puffy and could use to lose some more water weight, which would be helpful on his lungs and heart too. He gets his diuretics 4 times a day for this, so hopefully they will continue to work to get this excess fluid off of him. Overall, he is doing remarkably well for what he has been through and in comparison to where he was just 4 weeks ago. At that time it was looking quite grim, and we weren’t sure he would be able to pull through, but look at him now. We are so blessed. We want to share the sad news of Juan’s passing this past Friday night in PICU. After a VERY, VERY long fight against his cancer, his battle is now done. We pray for his family who now grieves his loss, asking God to bring them peace. We continue to pray for Manny, Hermes, and John Evan who remain in the PICU, still very sick. May God bless their sweet little bodies with healing, and their families with strength and peace. We continue to pray for all of the other PBMTU kids/families, Krabbe kids/families, and those who have lost their children to such horrible diseases. God bless them all. Thank you for all of the prayers. Thank you Kelly for sitting with Noah so that we could go out to eat with Bobbie, and thanks Bobbie for dinner! It was great fun and such a treat! We are so blessed to have such great friends.

Friday, June 20, 2003 9:46 PM CDT

Entry 124—June 20, 03/ Post Transplant Day 121/ Post Transplant Boost Day 58

Noah had a quiet night, maintaining good blood oxygen saturations on just ½ L of oxygen through his nose. His chest x-ray though, now shows some cloudiness in his right upper lung and in his left lower lung. We have had to suction him every 2-3 hours to try to keep his airway clear of mucous. We have been trying to turn him every 2 hours and do some chest physical therapy to help keep his lungs open. We are so pleased and delighted with how well he is doing thus far, but we (or at least I am) are in a constant state of worry as to how much longer he will be able to maintain himself. Will it be days, weeks, maybe even months? Will we be able to take him home via Medivac to Hawaii so that we can enjoy this time together as a family in our own home or left to finish his time here at Duke in this little 10 x 10 room? The plan is basically just to wean his sedation and pain medications and increase his tube feedings; and to just follow Noah’s lead. We are very grateful for all of the wonderful prayers that are being said, for the many of you who have spread the word that he is in such great need of prayer right now, and for the wonderful and encouraging words we have received via email, guest book entries, cards and packages. We continue to humbly ask for your prayers for Noah as he faces these uncertain days (weeks, months??) ahead. Thank you. Please also remember Hermes, Juan, Manny and little John Evan in PICU; and the other PBMTU kids, Krabbe kids, kids in outpatient and those parents who are now without their children. God bless them all.

Thursday, June 19, 2003 4:34 PM CDT

Entry 123-June 19, 03 Post Transplant Day 120, Post Transplant Boost Day 57

PRAISE BE TO GOD!!! Noah extubated with no problems and is doing great. He is breathing on his own, with no support but only a little bit of oxygen, comfortable, eyes open, pink and warm. We are still deep in the woods, but there is some light coming through the trees. All this is awesome but then it got better. Dr. K handed us his donor cell report. I stared at it for what seemed eternity, reading over and over, my mind not believing what my eyes were telling it. Noah is now at 14.5p from 10 His hand has definitely moved over this child, and for this we are thankful. We still can’t hold him but we are together as a family in our own room. We will continue the vigil; we will continue to pray, we will continue to have faith. In peace through Christ. Hope all enjoy the pictures of Noah in his new room. Thank you for all of the wonderful support and prayers. God has certainly been listening, and we pray that Noah continues on a path towards healing…surprising everyone. God bless.

Wednesday, June 18, 2003 10:54 PM CDT

Entry 122-June 18, 03 Post Transplant Day 119, Post Transplant Boost Day 56

We have had a long day. Noah could not be extubated because they thought he had swelling in his airway and would not have been set up for success. We waited all day for that decision and it was agonizing; but it is in Noah’s best interest, and if one more day increases his chances of success, then of course we are all for it. As we sat at his crib side holding his hands, because even though he no longer has an EVD we still can’t pick him up because he is intubated, I told Debbie I felt like we were on death row. It is a uniquely terrifying feeling, like nothing either of us has experienced before. As the time ticked by, and we pulled the curtains so we could have some quiet time with Noah, we began to experience some peace and solitude. Noah looked incredibly beautiful today, serene, like an angel, so soft and warm, his little mouth gnawing on his breathing tube, we could only sit in awe and silence and stare at his splendor. It was stormy today, but for a while the sun came through the clouds and shined on Noah’s face, he glowed in this light, it bathed him entirely. What a perfect creation God has made, how lucky we are to be this child’s parents, how hard it is to imagine losing him. We are at peace…God has given us this. We will love him by seconds, and hope for hours, that lead to days. They will see how he is doing tomorrow morning and extubate him, we will then be going to the bone marrow floor, and hopefully we can finally hold him. Thank you for all of your prayers and support. We will do our best to keep everyone updated. What are these clouds that gather, clouds of sorrow or clouds of life? We can only watch in wonder, for only God knows the answer.

Please, as you are on your knees praying for our sweet Noah, remember Juan, Hermes (brother of Aristophanes who passed just weeks ago in PICU), Manny and John Evan, who are all so very sick and reside in PICU with Noah. Please continue to remember their families in prayer, as they deal with the anguish of sitting at their child’s crib side facing their own difficult trial. We continue to ask that you remember the other PBMTU kids/families, those in outpatient, Krabbe kids/families, and those who have lost their child to such diseases. God bless them all. Thank you.

Tuesday, June 17, 2003 11:45 PM CDT

Entry 121-June 17, 03 Post Transplant Day 118, Post Transplant Boost Day 55

I write this journal with many mixed emotions. Noah has remained stable on low ventilator settings after successfully having his shunt internalized today. He is doing well, I guess, when looking at him from the outside. In fact, he looks much, much better than he has in a few weeks. We are so pleased to have him back down to his ‘normal’ size and doing so well on his ventilator settings. His chest x-ray looks much improved from last week, clearer in both lungs and his right lung appears to be fully opened up. He is still having quite a bit of mucous, and is being suctioned every few hours via the breathing tube. We have not been able to hold him yet, since he remains intubated on the ventilator, but maybe when he is extubated, as long as holding him does not make him uncomfortable and agitated. Dr. K stopped in after he returned from surgery to tell us that tomorrow they will look at trying to extubate him, probably in late morning to early afternoon. We will then move him back over to the bone marrow unit…not our old room of 5205, but it doesn’t matter. After being in the PICU almost 4 weeks, we are definitely relieved to be able to roll him back over to the PBMTU, because that doesn’t always happen, thus we know that we are blessed. However, we have come to a decision; we are going to let Noah fly on his own. We did not want to have to make the decision of whether or not to withdraw life support, so we have requested that the docs get him in the best possible condition to allow him the best opportunity for success upon extubation, in hopes that he will do well. This will allow him to decide this for us. If he cannot do it, then we have chosen not to intubate him again, but to attempt to keep him comfortable with oxygen, medications and lots of cuddling. We will allow him to speak for himself. God will make His Will known, and we will finally have an answer to what we have been waiting for, for quite some time. This is so very difficult on us, but we know that we must do it this way. Thank you for your continued support, but we ask now, more than ever, that you will lift us up in prayer as we face some very dark hours ahead. Noah has always been one for surprises, but we also know that his little body is so very tired, and he is not the same baby we brought to Duke in January. Sometimes in life windows open to us, if just for a moment. And we pause at the windowsill, unsure… unable to leap through them. In a moment, as quick as the window opens, it slams shut and we never know what would have happen had we took a chance moment and jumped. When we dive through open windows on opportunity, the endings can be terribly tragic or miraculously wonderful. The falls can be great or small the landings painful or pleasurable. We saw the window open briefly, and not wanting any regrets, we took the leap as a family, a leap of faith. It happened so quickly…sudden…unexpected, that we left all our baggage behind. Decided that this window could not be allowed to shut, to leave us with a lifetime or regrets and wonders. We are still falling; this journey was longer than we could of ever imagined. Sometimes great rewards require huge risk. And so, sometimes, when windows open, you have to jump, least they are closed to you forever. The ground is approaching fast now. We are thankful for all of you who will be there to catch us, as you have always been. Peace be with you…

Monday, June 16, 2003 9:08 PM CDT

Entry 120—June 16, 03 Post Transplant Day 117/ Post Transplant Day 54

I will not waste the space on Noah’s journal to address someone’s guest book entry from today; all of our family and friends did a fine job. I will say, that over the months, we have enjoyed all of the encouragement and prayers given us through your guest book entries. When things have looked bleak all of you have been there to give your support, prayers, and comfort. We have tried to give you a glimpse into our lives and have always strived to be open when making entries, even though we know openness can leave you vulnerable. So thank you, all of Noah’s most dedicated fans, for your continued compassion. Noah has done fine today. Still on the ventilator but steady. Tomorrow his shunt will be internalized and after he is extubated we will finally be able to hold him again. We can’t wait, we have waited three months, it will be like Christmas in June to finally be able to pick him up and hug him tightly. We will give him a big hug from all of you. Please continue to pray for all families and children fighting illness and in some cases ignorance. Peace

Saturday, June 14, 2003 10:19 PM CDT

Entry 119—June 15, 03 Post Transplant Day 116/ Post Transplant Day 53

Debbie left the Father’s Day entry for me. I am at a loss for words; the last three weeks have been so hard on all of us. All I can say is that every time a child is born there is always a “Father”. Many times, for whatever reason, children grow up and never know their fathers. Children may be abused, neglected, and unsupported by fathers who fail to meet their responsibilities to their children. For that reason, I would prefer to be called “Dad”. My father was always Dad to me. He made sure to be at all my school activities, picking me up when I feel down, giving me guidance with understanding, disciple with love, and encouraged me to be the best. He made mistakes, no parent is perfect, but he always acted out of love, and in what he thought was my best interests. So to me he is Dad. That is all I want to be to Noah, his Dad. I am so proud of my little boy, and could think of no greater pleasure than to be his Dad. I want the best for him, to protect him, to comfort him; he deserves nothing less. What I can do for him now is love him like there is no tomorrow and trust the rest to God. I will never forget this Father’s Day, nor will I forget that any man can be a “Father”, but that you have to earn the right to be called “Dad”.

Saturday, June 14, 2003 4:14 PM CDT

Entry 118—June 14, 03 Post Transplant Day 115/ Post Transplant Day 52

I have been trying to muster up some creativity to write a more thought provoking, heart tugging message in celebration of Father’s Day, but writing is not really my forte’. I have surprised myself with a few entries in the past…but I usually leave those journals to Greg. He has much more creative juice flowing through his brain than I. I know just how special Mother’s Day was for me, and I know that this holiday will be quite special for Greg in a similar manner. We have learned to try and live each day as though it is Noah’s last…making sure he is told just how much he is loved and that there are so many people praying for him. These particular holidays (Mother’s & Father’s Day), falling just around this tragic time in our lives, seems to truly emphasize to us the love felt between a child and his parents. More so than words could ever express, and yet it seems that it is realized and appreciated even more so when threatened by illness, death or separation. We have been truly blessed to be Noah’s parents and to be able to show him how much he means to us and how much he is loved each day. I guess my point is that I view these holidays as not only an opportunity to recognize Moms and Dads for doing such a great job, but also for moms and dads to appreciate being blessed with this ‘job’. We want to openly say how thankful we are for being blessed as Noah’s parents. Often we both wonder ‘Why us?’, as it would be a lot more fun and less painful on us all if Noah were ‘normal’…but God knows best and we are learning to just give thanks and pray for his help daily. We wish all Dads a very blessed Father’s Day, saying ‘Thank you’ for doing such a great job, but also asking that Thanks be given to God for the privilege of being a Father.

Okay, I am off my soapbox and now on to giving Noah’s update. He remains stable, on the ventilator and waiting for next week’s events. We might have some preliminary word on his MRI results this evening from Dr. K, but most likely next week. I must admit that I am terrified of getting this news. I suppose it is similar to the feeling I had when waiting for the test results after taking my State Nursing Board Exam…not sure what to think, excited for the best, fearing the worst. If he has brain stem damage or significant progression in his disease, then this will be a huge determinant in making some of our final decisions in his plan of care. I keep trying to tell myself when I start to worry that God knows best and is in control, but sometimes God’s plan is to take children and not heal them…and although I know that Heaven is far better a place than Earth…it is terrifying and extremely painful to think of letting go of Noah. We wait anxiously to hear the news and praying desperately for his healing. Dr. K is on now and we are so relieved to have her so close by. She has some tricks up her sleeve for Noah’s care that might be helpful in getting him off of the ventilator successfully next week. They will wait to extubate him until after his surgery on Monday/Tuesday, so we pray that this all goes well. Thank you so much for all of the prayers and support. Thank you so much to Courtney Hurd and the members of the Hendrick Motorsports Team and Hendrick Marrow Program for the visit to the PBMTU and the wonderful gifts for all of the kids. Noah’s afghan is beautiful and his little “Prince in Training” Hat is adorable…was that from Robin Tucker? Thank you! Thank you for all of the racing gear…Greg loved that!! For those who are not familiar with the Hendrick Motorsports Team, they are the NASCAR racing team that includes Jeff Gordon, Terry Labonte, Jimmie Johnson and someone else whom I have forgotten (sorry). This also includes the pit crews and many others. Thank you to all of you for supporting these kids/families. It truly means so much. Thanks for remembering all of the other PBMTU kids, outpatient kids, PICU kids and Krabbe kids in your prayers too. Juan and Manny remain quite sick still. Little John Evan is making some small improvements…but remains very sick. Happy Birthday to our little friend Trenton Alley, who just turned 3 this week!! God bless.

Friday, June 13, 2003 6:37 PM CDT

Entry 117--June 13, 03 Post Transplant Day 114/ Post Transplant Boost Day 51

Noah had a stable night, which is so nice after such a rough couple of days. We were able to get back to the hotel and try to get some ‘good’ sleep, but even that has become difficult. Noah had an MRI today, which will give us a major piece of information about his disease progression…I have been anxious all day just wondering what we are going to learn. We have a lot of tough things to think over now given his situation. If his MRI returns showing brain stem damage from his Krabbe progression, then we will need to know how his cells are doing. They will draw another sample on Monday morning, which will again take a week to return. We should know about a week from now what his engraftment is doing or not doing. If he is losing his donor cells, then coupled with the MRI results if they are not good, then we must decide whether or not it would be beneficial to pursue a tracheostomy. If he loses his engraftment and has brain stem damage, then doing this procedure would only allow us to keep his body ‘alive’ on a home ventilator while he fades away…later having to decide when to withdraw life support. We do not want to put ourselves into this position…and we won’t do it. It is not fair to any of us. We have been through too much pain as is to endure anymore. On the flip side, if the MRI comes back with little to no progression of his disease and basically no brain stem involvement, then again we will wait for his engraftment results next week. If his cells are still at 10% or climbing higher (which would be ideal), then we would definitely consider possibly doing the procedure. At this point there are so many “What if’s?” and we really need some hard data to look at. Dr. K will be on call starting tonight for the next two weeks and this will be extremely helpful to us in trying to sort all of these issues out. For now, he is stable on low ventilator settings, just hanging out in the PICU…and waiting for Monday/Tuesday to have his shunt internalized FINALLY after about 3 months. We are so blessed and it is a miracle that it has not gotten infected. It is a 1 out of 3 chance for infection for the EVD for those with normal immune systems, and he has done very well despite his being immunocompromised. We are pleased to know that it will finally be internalized. He has lost quite a bit of fluid over the last week, and looks incredibly better. We will try and take some more pictures of him, but I think they kind of frown on taking pictures in the PICU even if it is only for his journal. We will try and do our best since it has been a while. Thank you for all of the prayers for Noah. We cannot imagine going through this and not having all of this tremendous support, encouragement, and army of prayer warriors backing us up each day. We pray for you each day at Noah’s bedside, God bless you all. Thank you to those other PBMTU families for including us on your prayer lists and web links. Please remember to keep Manny & Juan in your prayers, they are also bone marrow kids in the PICU right now. We also would like to ask that you remember a little baby named John Evan, who has an unknown fatal Metabolic Disease and is in the PICU fighting for his life. His family is so wonderful and loving and has showed us so much support and kindness. We ask that you will remember this family in your prayers too. Please continue to pray for Noah’s healing, as well as for the other kids in PICU, PBMTU, outpatient clinic and all of our Krabbe kids. One last thing, we would like to wish our dads, Jon and Mark, and all of the other Fathers out there a wonderful and blessed Father’s Day. God bless.

Thursday, June 12, 2003 1:44 PM CDT

Entry 116-June 12, 03 Post Transplant Day 113/ Post Transplant Boost Day 50

I am glad that I did not update last night as I had planned, because I would have had to follow up yesterday’s good news with bad news…isn’t that the way it seems to always work? How ironic? Yesterday around 1600, Noah was extubated and placed onto a face mask that gives him oxygen with a little extra pressure added, known as BiPAP. He was tolerating okay, and improved into the early morning with good blood gases. We left his bedside around 0400 this morning finally somewhat satisfied that he was ‘all right’ and was going to get some sleep. At around 0900 this morning, we received a phone call in the hotel that Noah was not tolerating the BiPAP and his upper right lung had collapsed some, and he needed to be reintubated. When we arrived at the PICU around 0930, we were met by the bone marrow team and social worker. We used to think that something was up when the social worker would hang around, but after awhile, she seemed to always be around each day anyhow whether or not we were receiving bad news. So we did not think too much of it until we were asked to go into the PICU conference room to sit down. Yikes!! What news were we getting, because good news is always given standing up, not having to sit down around the conference table in private with the social worker. Well, the news is that the bone marrow doc on call right now and the PICU docs think that Noah got into this predicament because he no longer can handle and swallow mucous, and thus his airway gets clogged and he gets into distress. Therefore they are highly suggesting as a last resort or last intervention for Noah to have a tracheostomy done. This means that he would be on mechanical ventilation, even when we leave the hospital. We don’t believe that is what put him into this situation. He was basically on ‘room air’ and not needing any oxygen or assistance breathing the morning that he eventually came over to the PICU needing ventilation. He began to dramatically swell and have serious fluid shifts and then this caused him to have difficulty effectively oxygenating, which led to his getting distressed…but he was not having trouble with mucous until his weight had already increased by ½ a kilogram (basically a pound) within a few hours. We are at a loss as to what to do or truly believe. We know that these docs mean well, but they are the same ones that were indirectly encouraging us to throw in the towel because Noah was/is a lost cause. Noah is not on high settings on the ventilator and could easily be weaned off again, bearing that his right upper lung clears up and his fluid continues to be lost. We have other issues right now that we are working on, our plates are absolutely topped off right now, and we cannot take much more. Dr. K is out of town until tomorrow night, and then she will be covering on call for the next 2 weeks inpatient, so we will be able to see her everyday to personally discuss Noah’s plan of care. Please keep him in your prayers, he needs so much prayer right now. Thank you.

Tuesday, June 10, 2003 6:16 PM CDT

Entry 115 June 10, ’03 Post Transplant Day 111/ Post Transplant Boost Day 48

Noah is doing well from his surgery yesterday, he had his EVD replaced with another external drain…again, but if this one does well for him this week without any signs of any possible infection, then maybe the first of next week he can have it internalized into his heart permanently. The neurosurgery team just wanted to be certain that there was no infection present, so they decided once again to leave it external for now. He was taken off his pain medicine early this morning and his tube feedings stopped in preparation for extubation today, however, he began developing withdrawal symptoms from the pain medication (a narcotic) and was having high blood pressures, a high heart rate, and very high respiratory rate, and he was having jerky, spastic type movements all over his body throughout this morning. He was definitely very uncomfortable. So his pain medication was restarted at half of what is was when it was turned off, but this did not seem to help him much. After a lot of heavy pushing of the doctors, we finally were able to get him back on the regular dose of pain medication, and then have some Methadone and Valium started for his withdrawal symptoms. Then after these two medications are in his system, then they will gradually wean him from his pain medication slowly, preventing him from having serious withdrawal symptoms. He is now more comfortable and seems to be feeling better. He remains on very low ventilator settings and doing relatively well. Hopefully he will be able to be extubated in another day or so. We ask that you will continue to remember him in your prayers, along with Juan and Manny who are also in the PICU from bone marrow. Please remember all of the other PBMTU kids, bone marrow kids in outpatient clinic, the Krabbe kids/families, and those families who have lost their child/children to these terrible diseases. Thank you so much for all of the prayers. They are certainly working, because Noah is still here with us, and fighting harder than ever to survive all the terrible things that have been thrown his way. God bless.

Sunday, June 8, 2003 11:51 PM CDT

It is hard to follow up these last few journal entries…Greg is truly doing a number on my heartstrings, but this will be brief since it is so late and we have to make an early trek to the PICU waiting area to meet with the neurosurgeon in the early morning. Noah is going to either have his current EVD replaced with a new one, since they are only supposed to be good against infection for 7-10 days and he is way overdue, or he will have his shunt finally internalized. We won’t be certain until tomorrow when we sign consents and speak with the doctors. As for his update, Greg was supposed to share with you that Noah’s donor cell test returned on Friday with his engraftment remaining steady at 10%, no more, but no less. We all, including the docs, don’t really know what to make of this and where it will be next time, but they will test him again in another week for any changes. Of course we are praying that God will bless him with an increase in this number, but are so very thankful that he has blessed him with no decrease in it this week. We owe a huge heap of thanks to God for blessing Noah this week with huge steps towards healing!! Noah is now on ventilator settings that are acceptable for extubation, however, he still needs to lose about a liter of fluid before they will feel comfortable about taking his breathing tube out…so he is still intubated on the ventilator for now. BUT…that is okay since he has to go to surgery in the morning and will have to be intubated anyhow. Then he will return to PICU and once he has been able to recover from surgery then they will likely try to wean him again and then extubated. As of right now he is not opening his eyes yet, but he his gnawing and sucking on his breathing tube with his little mouth, occasionally slightly moving his head and feet. He is now being tube fed again through his gastric tube at 10cc/hour (2 teaspoons/hour) and is tolerating it well. He was weighed last night and is up to 9.6 kg (not sure what it is in pounds, but 1 kg = 2.2 pounds) and his weight prior to going to the PICU was around 7.6kg. So he needs to lose a lot more fluid before he gets back to where he was. He has been losing on average about 1/5 of a liter each day, so we are thinking that by this weekend he might be able to go back over to the bone marrow unit. We continue to pray for complete healing, that he will have a successful surgery tomorrow, he will soon be extubated and breathing well on his own, that he will not have any more problems with fluid or infection, and that his donor cells start to grow for him. Whew, that is a lot of stuff, but God is able and we are praying and having faith that he will completely heal Noah. Thank you to everyone for all of the prayers for Noah, we know that it is your prayers that have helped Noah to get where he is, along with God’s grace and mercy. We cannot say enough thanks to each of you for all of the support and encouragement and prayers and financial help that you all have provided us. We cannot begin to remember all of you by name, but THANK YOU!! I do want to mention a special Thank you to Kapi’olani PICU staff for all of the AWESOME support you have provided from so far away. You all are truly the epitome of ohana, and I am so blessed to have your support! I cannot wait to get home and get back to my “normal” (?) life! Thank you to Greg’s unit for allowing him to stay here and fight for our sweet baby’s life. He has been essential to Noah’s care and to me keeping my sanity! Thank you! Thank you to Moana and the Mailo family back home in Hawaii for watching over Yzerman, Makana and the fish, and for collecting our mail. THANK YOU SO MUCH!!! Thank you to all of the 5200 nurses who have taken such great care of us all, as a family. We owe so many more thanks to so many others, including many of our fellow bone marrow families. Please remember to keep them all in your prayers, including the kids/families on 5200, in outpatient, and in PICU. We want to wish the Miles family a big Congratulations for the arrival of little Degan Cain Miles, born this morning and will be transplanted in a couple of weeks for Krabbe’s here at Duke. We wish them the best. We ask that you lift Juan and his family up in prayer, as he is not doing very well at all. Please remember to pray for all of the doctors caring for these kids, that God will lead them and they will listen. God bless you all, family, friend acquaintance and stranger alike, for all of your support.

Saturday, June 7, 2003 8:29 PM CDT

Entry 113-June 7, 2003 Post Transplant Day 108/ Post Transplant Boost Day 45

Today was a bleak and rainy day in North Carolina. The storm added a touch of gray to the weekend quiet of the PICU. We continued the vigil at Noah’s crib side, oblivious to the weather. The view from his window, as the rainstorm passed over, could not dampen the feeling of spring in our hearts. Noah looks so peaceful today, wrapped in the blanket that was knitted by his mother and grandmother, completed only hours before the dangers of two weeks ago. This thick mantle holds him tight and warmly because it was made special for him, with loving hands and gentle touch. Peeking out from under the weight of the blanket, nestled snuggly under his arm, is his ever-present friend, Elmo. This furry red companion has been with him through every crisis, and has made three trips to the PICU. Several doctors walked by today and broke a smile at his ever-decreasing ventilator settings, happy to see the fruits of theirs and Noah’s labor. Today was a bright day after all, the sun did shine in room six, and the storm clouds were parted, all by the power and persistence of a little boy. Wrapped snuggly in his blank, cradled in the arms of Jesus, a testimony that miracles do happen, no matter what the weather looks like outside.

Saturday, June 7, 2003 0:53 AM CDT

Entry 112-June 6, 2003 Post Transplant Day 107/ Post Transplant Boost Day 44

When you look at the photo of Noah you see this small boy, puffy, weighed down with tubes, lines, and wires. It can be overwhelming at times, and when he first went into the hospital it caused us some distress, after all, no parent wants to see their beautiful child covered with tubes. It took us some time to except all of the lines and tubes as a necessary evil required for a better future. When a line or tube was removed it was always celebrated as a victory, a sign we were one step closer to going home. Now, Noah has infinitely more peripherals then ever before, and is in considerably more trouble. Sitting at his bedside tonight I realized something. His lines didn’t matter anymore, they were like shadows cast on his little body, present but transparent. I could see his strength and beauty like never before and it was uplifting and inspiring. I was brought back to the night he crashed, when for a moment he opened his eyes suddenly and looked at me. It amazed me and I called out, “His eyes are open.” The PICU team’s reply was to increase his sedation, sedation that was already pretty high. I will never forget that moment, it is burned into my memory forever, because for a while, I thought it would be the last time I would ever see his beautiful blue eyes. Now I know differently, he looked at me to say, “Don’t worry I’m not going to give up.” I am thankful for that look, it meant so much to me, but it also haunted me as we tried to sort through the cause. Now I see him like I never did before. He amazes me with his strength and has surprised all the doctors with his perseverance and recovery. Today Noah is breathing, still on the ventilator but only at a set rate, a rate he has been continually breathing over all day. His labs are perfect, and he continues to have negative fluid output. He has amazed the doctors, but not his parents. We know that we have been blessed with a beautiful child, and we know this because we alone see him like nobody else.

Thursday, June 5, 2003 11:11 PM CDT

Entry 111-June 5, 2003 Post Transplant Day 106/ Post Transplant Boost Day 43

This will not be a poetic recitation of Greg’s creativity, but a simple update of Noah’s progress. As I read on the guest book, I see that some of you have received bits and pieces of Noah’s progress via other caringbridge web pages of our fellow bone marrow transplant friends. Yesterday morning Noah was switched from the oscillator onto the conventional ventilator and has done well since, with a low oxygen requirement. His sedation is being weaned slowly, so he most likely won’t be awake enough to attempt extubation from the ventilator until this weekend. However, this will allow him more time to diurese some more fluid that he has. He has successfully managed to get rid of some each day and his kidneys are still doing well, despite all of the diuretics that he has been getting, which are known to be quite harsh on kidney function. His blood pressure has been relatively elevated for some time and is requiring a special high blood pressure patch and constant IV drip to keep it down. This could be from a few different causes, but as long as it does not get out of hand, he is all right for now. He had another donor cell test sample drawn Monday and we should receive the results from this tomorrow sometime. We are praying for the best possible results for Noah, which would be a donor cell percentage higher than 10%. This would indicate that they are still growing inside him and he has not lost his engraftment. He is so very puffy still, but is looking better with each new day. He will likely have his EVD replaced again in the OR within the next few days…AGAIN! BUT…if he doesn’t then there is a risk for infection. We pray that he will eventually have it internalized permanently real soon. He will likely start his tube feedings again very slowly, so we are hoping that he tolerates them well. His belly is so bloated with fluid that it seems impossible that it will tolerate much more being pumped into it, but we are praying that all goes well. It is truly amazing to see his improvements over the last week. For those of you trying to contact us via phone to Noah’s bone marrow room, then I am positive that you have had no luck in reaching us. We were booted out yesterday, because they might need the room and need an open one just in case of an admission. We did not really want to bother trying to move everything out since Noah WILL be moving back into it soon…but we understand the reason for this and it was no big deal since Noah’s improvements have way overshadowed this minor issue! So, if you are trying to reach us, we can be reached via email, cell or in the hotel (only when we are not in the PICU with Noah).

We can only give all glory and honor to God for these miraculous improvements that Noah has made. What a true blessing! We want to say THANK YOU a million times over for the large abundance of prayers that have been said for Noah, and the other kids. They are working! Please continue to remember Noah in your prayers that he will continue to improve and wean off of the ventilator, continue to lose all of that fluid, he will not have any further trouble with fluid or infection, he will continue to grow donor cells, he will tolerate tube feedings again, and that he will have his shunt internalized again soon. Please remember baby Jordan Horton & Juan (bone marrow kids in need of some special prayers), kids in outpatient, Elly and Maddie (Krabbe non-transplanted kids that need prayers), all of the other PBMTU and Krabbe kids, and those families who have recently lost their children.

Wednesday, June 4, 2003 2:50 AM CDT

Entry 110-Jun 4, 2003 Post Transplant Day 105/ Post Transplant Boost Day 42

The PICU is a mystical place, quiet but alive with the ever constant hum of machinery. All around parents hover over their children in silence, watching and waiting, for some sign of improvement. Some children suffer alone, and so you cannot help but suffer with them, and wonder why they must endure their pain without a parent’s comfort. The breathing machines whine and purr away, and every so often the calm is broken by the alarm of a medicine pump or oxygen sensor. Sometimes in the distance a quiet sob flutters through, but you dare not look from were it comes, least you be caught up in someone else’s sorrow. Your world quickly becomes one of numbers, the monitors like a magnet for your eyes, every change noted and recorded in your brain. I have often tried to tune the screens out, to focus on my son, but they always call out for attention. Night and day this goes on and you can begin to feel like the character in Poe’s, The Pit and Pendulum. But why so much torment? I explained to a doctor the other night, it is not my son on an oscillator, but our whole family, we ache with him, and his pain and improvements are ours also. For the families who reside on the bone marrow floor, going to the PICU is like crossing over the river Styx. The ferryman extracts a heavy toll, and we are aware that often parents are not able to pay it, and must leave their child on the other side. Once back to the unit the journey is not over. The trail you take, your child must lead you down, and it is often dark and full of danger. There are many shadowy corners and side trails that hold unseen perils. You grasp their hand tightly, because at any moment, from out of the darkness, can pounce a hideous monster that will snatch your child from you, and try to take him back…across the river. All of us, hope to make it to the end of the trail, clutching our children tightly in our arms, we look for the sun. Hoping to break out into green meadows, and finally come to rest under blues skies and soft breezes, basking in the warmth of the sun together, as a family. Today, Noah’s efforts have gone a long way to pay the toll back across the river. We anxiously await his hand to see where he will lead us next.

Tuesday, June 3, 2003 4:30 PM CDT

Entry 109-Jun 3, 2003 Post Transplant Day 104/ Post Transplant Boost Day 41

Jesus was walking with his disciples and they came across a blind man, and his disciples asked him,” Who had sinned, this man or his parents that he was born blind?” They asked that question because in the Hebrew tradition it was believed that children could suffer for the sins of their parents. But Christ new differently and told them that neither had sinned but that he was here to do God’s work. Here to do Gods work? This answer must of perplexed the disciples. What possible use could God have with a man who could not see? The handicapped of that time period led a miserable life, often dependent on the charity of others just to survive. So how could this poor soul do any work for God, he could barley feed himself? This unfortunate man, destitute and blind, would feel the hands of Christ on his face and see again, serving as a testament that Christ is the light of the world. God used this man, who would have never received a second glance in his society, for... a miracle. This passage has given me great comfort while we have been here at Duke. It speaks to me of my son, a gift on loan from Him, a child who has spent all but three months of his life in the hospital. Who has fought a rare disease, endured days of pain with no way to tell us, suffered the agony of chemotherapy, and looked death in the eye. He is my hero, braver then I could ever be, with the courage of an entire army. He has been around the world while confined to a bed, and touched many with his story. He has puzzled the doctors and forced them to look were they haven’t looked, and softened the hardest of hearts. He has changed my life and Debbie’s, nothing looks quiet the same any more. He has brought thousands to their knees in prayer, and made parents hold their children a little tighter, thankful for their gift of a healthy child. Noah has done God’s work, he is part of His miracle and I thank Him every day for such a precious gift, and the honor to be this child’s father. He has made Debbie and I whole and taught us what love is all about, strengthening our faith and that of those who hear his story. This child who can barley move, bed ridden and on an oscillator, beautiful and strong, a testimony of Gods love. You ask why has this happen to him? Who sinned, this child or his parents that he is so afflicted? I answer No…he is here to do God’s work.

Monday, June 2, 2003 8:42 PM CDT

Entry 108-Jun 2, 2003 Post Transplant Day 103/ Post Transplant Boost Day 40

I love my son, but just as important I love being his Dad. Noah and I have a special bond and though he can’t talk to me with words, he talks to me through other means. As I sat at his crib side, he told me something very important. He showed me that he didn’t have a virus, infection, or fungus; he showed me that he had inflammation. So what could possibly cause him so much inflammation that his entire body was reacting? I knew the answer had to be in a medicine that was delivered through his IV. I posed this question to the doctors but they were convinced that it was an infection and would not budge, and so I was left to figure out the mystery and take action, because Noah was only getting worse as time passed. So, at 0200am, I began to sort through his medications looking for anything that would explain some of the puzzles that had baffled the doctors. I was drawn to an immunosuppressant drug called FK506. This drug was given to him twice a day and was started in March around the time we saw major changes in Noah. I was able to obtain a complete write up on the drug and it hit me like a ton of bricks. When Noah crashed on Saturday his Hemoglobin dropped drastically to 4, which is dangerously low. One of the major side effects of FK506 is a sudden drop in hemoglobin. The next one was difficulty breathing. Noah’s respiration patterns had changed drastically in March and on Saturday they were at their worst. Next were vision problems, again, a problem that began around the time he was put on the medication; this is the only symptom we could get the doctors to admit they had seen. Next were sudden unexplained weight gains, we have had numerous incidents of this, all unexplained, that often have caused him problems with breathing because of fluid leak into the lungs. Noah blew up like a balloon on Saturday. The most severe reactions all came around the time his steroids were weaned, which would have allowed for more of the chemical to bond to his tissues causing more irritation. On Sunday morning he was given a steroid boost and we saw a dramatic improvement in his swelling, but on Monday a different doctor cut his steroids and he began to dramatically blow up over the next 24 hours, eventually having 6 liters of extra fluid. So, reading this information, and knowing that Noah, as tough as he was, was not going to be able to tolerate much more, I had to act to save my son. I knew that they were infusing him with FK506 twice a day and that his first dose was due early morning so at about 0300am I marched into the PICU and asked to see a doctor. I told her that they were quick to remind us that we needed to make the decision to turn off his oscillator, but instead we were turning off his FK506. She reluctantly wrote the order to stop the medication. They continued to treat Noah for an infection but within a little over 24 hours he began to lose fluid and the inflammation started to go down. Not until after two days of improvement did the doctors start to treat him for inflammation, and finally give him the steroids he needed. Noah has now lost almost an entire liter of fluid in the last three days and his lung x-rays are dramatically improved. They will not admit it was the drug that caused all this but it doesn’t matter, Noah is getting better. He is breathing much better now, and the gentle squeeze of my finger says “Thanks Dad”, as I whisper in his ear “I love you son.”

Saturday, May 31, 2003 6:32 PM CDT

Entry 107-May 31, 2003 Post Transplant Day 101/ Post Transplant Boost Day 38

What a terrible week this has been. We have once again been on the proverbial emotional roller coaster and it sucks!! We were grateful to have Grampa and Uncle here to offer support, but we feel so alone in our struggle. It is not that we are alone in praying for Noah’s healing, or that we haven’t received encouragement…we have and we are very, very grateful for that. However, we once again have to fight so hard for him. When this incident happened a week ago today, it came about so suddenly…and shocked us all, especially since he was doing so well, we had received encouraging news about his donor cells, and he was scheduled to have his shunt internalized finally. We have sat and thought, pondered, discussed and tried to come up with what we must have missed or overlooked. We have usually been able to notice signals before Noah is ever close to getting this critical…and we did notice that something was not right last Saturday afternoon, but he went downhill so fast. We had to make some tough decisions since we had Noah classified as a “Do not resuscitate” in his chart, but we of course just could not let him go without trying some intervention since he has fought so very hard to get this far. There has been a lot of discussion as to what brought this about…infection, lung hemorrhage,…and all we get is “we don’t know”, “it is too early to say”, “we will have to wait and see”. Well, it has been a week now and we still basically have no answers. There have been several cultures taken of his blood, mucous from his lungs, and spinal fluid from his drain to see if there is any bacteria, fungus or virus that he may have that caused this event. So far, nothing real significant has come up. There were some WBCs that showed up in a “cell count” from the spinal fluid sample, but the culture itself has grown nothing. It remains somewhat inconclusive for infection. There was one sputum sample that came back with a form of Strep bacteria, but was noted that it could have been a contaminant since nothing else has shown up on the other mucous samples. Therefore, it remains somewhat of a mystery since he has had no fevers or any positive blood cultures. What else could have caused him to have such a dramatic fluid shift that compromised his airway?? What about an allergic response to a medication?? His fluid may be leaking from his blood vessels because of inflammation due to an allergic response to a medication. When we approached the docs with this it was shrugged off as highly unlikely. Highly unlikely does not mean impossible though. They seem determined that he has an infection somewhere, and because of this firm belief we don’t feel that they are voluntarily looking into any other causes. Well, here we are now a week later, Noah still on the oscillator and stable, yes, but unable to be weaned any further because he is so very, very swollen with fluid. If they decrease the pressure on his oscillator, then it will allow the fluid that is sitting in his tissues to seep back into his lungs and once again put him into distress. He must first get rid of the fluid before he will truly be able to wean from the oscillator. Basically it appears that we are stuck, stagnant and moving nowhere. Unfortunately, Noah cannot stay on an oscillator for too much longer and he either must start making improvements or he will likely have great difficulty coming off of it. We recognize that this is everyone’s goal and that everyone has his best interest in mind, but it seems to us that some people have already written him off as a loss cause. I cannot begin to express how much this hurts…to know that we have all (Greg, Noah and myself) fought so very, very hard to get to this point only to have someone tell us in so many words that it doesn’t matter if he makes it off the oscillator/ventilator because he probably won’t engraft and will die anyhow. If we listened to the doctor who told us that we were overreacting and paranoid, then Noah would already be dead. If we listened to the doctor that told us that there was nothing that could be done to treat Krabbe’s Disease, then Noah would already be dead. If we listened to the doctor who told us that Noah did not have hydrocephalus and that it was just his disease progression or a vagal response, then Noah would already be dead. I could go on and on…but my point is that because we chose not to listen to these doctors and go against their professional medical opinion, Noah is still alive!! He is not dead yet…so why are we trying to treat him as if he is a lost cause and not worth the effort. That is crap! We refuse to sit at his crib side and mourn our son who is still alive and fighting. So this is what we have done. We looked up a few of his medications that he receives and there was one that he was changed over to in early March to help suppress his immune system. Since then it seems that he has had a lot of trouble with weight gain from fluid build up. If you have followed his journey then maybe you have seen some of the pictures in which he has been extremely puffy, and then others where he has thinned out again, and then puffy again, etc. It appears to us to have a puzzling pattern to it. Well, some of the side effects to this medication include swelling, unexplained increased weight, unexplained hemoglobin drops, difficulty breathing, rashes, blurred vision or visual disturbances, among others. Noah has had all of these, and maybe it is just coincidence, but then again maybe not. They still cannot explain the dramatic and life threatening drop in his hemoglobin last Saturday night since there was no bleeding found. We mentioned our theory to the docs and were basically shut down. Well, you know how the Prichard’s are…so we told them not to give this med to our son…we were refusing care...a big no-no, I’m sure some would say. Dr. K told us this was not wise, but she too is convinced that it is an infection. We don’t want his antibiotics removed…that would be stupid, because we could very well be wrong and if we are then he should be covered with antibiotics just in case. However, while we wait for his cultures to grow something and confirm the presence of infection, he keeps getting more swollen and remains on the oscillator, so why not try checking out other possible causes. They have changed this med to a different medication for us, which will do the same job, but works a bit differently. So we will have to wait several days to see if there will be any changes in his fluid status since stopping the one medication. If he did have an allergic response to it, by keeping it out of his system, he may be able to recover, have his inflammation decreased, which will stop fluid from leaking out of his blood vessels into the tissues. If it wasn’t, then I guess we will have to push them to find another cause. Maybe it is an infection, but we cannot afford to waste time waiting for cultures to grow something and have it end up being something else, that if we had found earlier and treated, then he could have already been off the ventilator and possibly back on the bone marrow unit. God has blessed Noah with tremendous strength and will to live. We will continue to fight for his sake, as God is leading us to do so. Noah will let us know when he is done, and we pray that God make his will known to us. I don’t know that I am prepared to say goodbye to him yet, but I let him know that he is in God’s hands and to follow as God leads him. If he is ready to go, then he must go. What a difficult thing to say. We continue to pray for his healing, believing that God has the power to heal him and spare his life…demonstrating his power to work a great miracle. We know that with God, all things are possible, including complete healing for Noah. So we continue our fight, leaning on one another to get through this, and looking to all of you to help lift Noah up in your prayers. Thank you for your prayers and support. At times we are discouraged, often by some of the people that are supposed to be trying to help save his life, but it is God’s voice that we are listening to. Please continue to pray for Noah, for his doctors, nurses and medical staff to never give up on him, and for us to keep our faith and hope. Thank you.

Thursday, May 29, 2003 7:09 PM CDT

Entry 106---May 29, 2003 Post Transplant Day 99/ Post Transplant Boost Day 36

Noah had a relatively stable day. Early this morning he had to have his settings increased a bit for high CO2 levels, but since then he has been pretty much the same…which is good. We are so grateful for his stability, but of course hope and pray for more. Noah remains extremely swollen, and his diuretics are known to be hard on the kidneys, so they have had to back off on them a little. They work best if the fluid is inside the blood vessels and not out in the tissues, but most of Noah’s fluid is out in his tissues, which is why he looks so extremely puffy. Therefore, the diuretic is not able to work as effectively as it could. As for his lungs, well his chest x-ray looks remarkably improved since having that plug removed yesterday during his bronchoscopy. That area of his right upper lung has opened up more and is getting better oxygenated. This is good too and we hope that there are no other plugs that arise. The plan is to wean him slowly and take their time. If you know anything about Noah, he does not like to be rushed. He had another abdominal ultrasound today because his belly was again a little bigger and harder than the last couple of days. It did show fluid in his abdomen, but no more than it had before really. So they did not intervene in that particular area. We are so grateful for the tremendous support and prayers, and ask that you continue to remember him, as well as the other kids. Thanks to those of you who hopped on over to Elijah’s page to offer support to him, his sister Alissa who has Krabbe (not transplanted due to her being too far progressed to transplant), and their mom Carolyn. Thanks to those that remembered Jose’s family. God’s blessings to you all.

Just want to add a quick update on Aristophanis and Alex. Alex seems to be doing better and might be taken off of the ventilator shortly and be able to return to PBMTU soon. Aristophanis took a very bad turn tonight, with a lot of bleeding into his lungs and is now on an oscillator. He is in very critical condition right now and not real stable. He desperately needs prayers, along with his brother Hermes, who was also transplanted at the same time on the PBMTU but his first transplant did not engraft and he will need another next week. Please pray for their parents and the boys. We pray that God will show mercy upon this family and step in to help heal these boys. Please, Please, Please pray for them.

Friday, May 30, 2003---0520 AM
We are very sorry to share that Aristophanis has passed early this morning in the PICU. We ask that you will please pray for his family and his brother Hermes who will be having his second transplant since the first did not engraft. They both have the same genetic metabolic disease, not Krabbe, but quite similar. Please remember them in prayer as they still have a very long road ahead, not only grieving the loss of Aristophanis, but with the second attempt to save Hermes' life. God bless and keep them and we pray for blessings of peace for this family. Thank you.

Wednesday, May 28, 2003 10:37 PM CDT

Entry 105---May 28, 2003 Post Transplant Day 98/ Post Transplant Boost Day 35

Another day is coming to a close and what a day it has been. We want to first offer how sincerest condolences to Jose David’s Family who is now mourning since his death tonight. He had such a battle, quite different in some aspects from Noah, but difficult just the same. We pray for peace and understanding for them tonight. Jose you will surely be missed! God bless you sweetie!

Noah had a long night of very low pH’s and high CO2 levels, which meant they had to increase his oscillator settings. So much for the weaning. He remains puffier than ever, but is responding some to a new diuretic. He had his bronchoscopy today. The pulmonologist said that in the right upper lung he was able to dislodge and suck out a brown mucous plug (old bloody looking) and then some fresher blood gushed out from behind it. His lungs are very frail and extremely inflamed/ irritated. They sent some samples to lab and we will have to wait a few days for the culture results to see if anything has grown. The preliminary gram stain results indicate most likely no sign of bacteria or fungus, but no word on the viral gram stain yet. Again these are just preliminary results. However, by the evening his blood gases showed that his CO2 levels were below normal and his pH’s were higher, which means they were ‘over’ ventilating him and could come down on some of the settings. This is good news. Dr. K came by today to offer support and said that she thinks he should remain on the oscillator for several more days, since it is actually less damaging on the lungs than the conventional ventilator because of the way it works. They are planning to wean him a lot slower this time and not rush him as much. We hope that he can heal and get better soon. However, we then must worry if this has caused him to lose his 10% donor cells…because they have had to manipulate some of his medications that suppress the immune system, including donor cell growth, and this may cause him to lose what he does have. We will have to wait and see until they do another test. As well, his EVD still has to be internalized and one of the neurosurgeons has already declared that he refuses to internalize Noah’s shunt for at least several more weeks. Ugh!!! We are pleased at Noah’s ability to not get any worse and regain some of the very small improvements that he had lost the last two nights. We feel very blessed to still have the chance for him to get better and for things to work out the way we hope they will. He is still here and is still fighting tooth and nail for his life.

We want to say thanks to Grampa and Uncle for stopping in to see Noah and offer us support. We wish them a very safe journey home tomorrow. Thank you for all of the prayers and tremendous support. We appreciate the very encouraging guest book entries…even from folks that we don’t know or don’t leave a name. We appreciate the prayers from those who don’t sign the guest book, but are still remembering Noah in prayer. However, it is nice to read the entries and is uplifting, especially during this time. Thank you to the other families who have made mention of Noah and his need for prayers on your own caring bridge pages. We continue to ask for your prayers for Noah’s healing, for the other PBMTU kids (Alex and Aristophanis in PICU too), Krabbe kids/families, and Jose’s family. There are many kids that are in the outpatient phase that need prayers too. Please remember to check in on Noah’s Krabbe buddy next door to him on the bone marrow unit, Elijah, at his web page www.caringbridge.org/il/elijahbarron He and his mom, Carolyn, need a lot of support too. Thanks and God bless.

Tuesday, May 27, 2003 8:34 PM CDT

Entry 104---May 27, 2003 Post Transplant Day 97/ Post Transplant Boost Day 34

This is our second entry for today, but if you noticed the time, the first one was placed on the web page in the wee hours of the morning and this one is nearer the close of the day. Noah had little trouble last night keeping his carbon dioxide (CO2) levels down in his blood because he was not able to breathe it out of his lungs, despite the oscillator. So, since CO2 build up is acidic, then it can start to cause a lot of damage and the body’s pH begins to drop into an acidic range. This can be deadly if it gets out of hand. One way to alter this is to change his oscillator settings to try and pump more CO2 out of his lungs. The thing that was blocking it seemed to be fluid build up of some sort in his lungs. He is extremely swollen all over, even more so than yesterday. So his pressure levels, which he was weaning down on successfully, had to be raised again. By late morning he was requiring more oxygen and higher pressures to maintain adequate oxygen in his blood, to blow off enough CO2 to prevent him from getting acidic and to maintain a somewhat normal pH. It appears from his x-ray that part of his upper right lung had begun to collapse due to the excess fluid building up and pushing in on the little air sacs in the lungs, preventing him from receiving adequate ventilation. He did however have his high blood pressure medicines turned off and has been able to keep it down on his own so far. However, if they cannot figure out a way to solve his excess fluid problem then he may not make it off the oscillator and will die. The diuretics that they are giving him are not doing the trick to get rid of the excess fluid, which means that it might indicate that the source of the problem is indeed some sort of respiratory infection…unknown at this time. We are desperately praying that he will make it through this safely, but at this point it remains unknown. They might do a bronchoscopy, where they stick a microscope type of tube down into part of his lungs to see what is going on. He remains very, very sick and we are very scared of the quite possible outcome. We know that if anyone can do it, he can, because of his incredible strength and will to live. However, over time after having to go through so much, his little body is running out of strength…and his will may not be strong enough to see him through. Oh, I just received word that they are once again having to go up on Noah’s oscillator settings, because he is not doing well. We continue to ask for your prayers, not only for Noah but also for the other bone marrow kids in the PICU: Alex, Aristophanis, and Jose. We want to congratulate Madison and Brent on their jailbreaks from the PBMTU this week. Brent has been waiting a very, very long time for his discharge and has been such a trooper. We wish them both the very best. We ask that you will remember the other PBMTU and Krabbe kids in prayer too. Thank you so very much for all of the wonderfully uplifting guest book entries these last few days. Thanks to Amye & Mikhail for the care package. We owe so many more thank you’s to so many more people and we are sorry if we have forgotten to mention you, but “Thank You” very much. God bless.

Tuesday, May 27, 2003 0:35 AM CDT

Entry 103---May 27, 2003 Post Transplant Day 97/ Post Transplant Boost Day 34

Noah remains on the oscillator. Dr. K came by yesterday morning and said that it is a good sign that Noah is stabilized and able to adjust to slow weaning on his oscillator settings. He remains very sick, his lungs cloudy and not sure of the cause, his abdomen very enlarged and unsure why, and he is very swollen all over his body. At times his electrolytes and blood gases are stable, and at other times he is unstable and requires intervention. He is holding his own though and we are relieved despite his very critical condition. We have posted some pictures of him in the PICU, so this is your warning that they do not hide in any way what he looks like and is enduring. This is our reality, and if it is painful for you to see a picture of, well…it is what we see all day and night, and what Noah is personally enduring. But for some of you who have been in this same situation with your child, it may be especially difficult and you may not want to look at his photos. Grampa and Uncle Jon are here visiting from Missouri until Thursday. We hope that by then they will witness some more improvements for Noah. We cannot express enough how unbelievably touched we are from the enormous amount of support that everyone has given. The guest book entries have been phenomenal. We certainly appreciate the encouragement and the declaration of prayers for Noah as we experience yet another huge setback. Thank you so much. We are praying that Noah will improve enough to be placed back on the conventional ventilator by tomorrow sometime, and eventually extubated and on his ‘final’ road to complete recovery. Well, we can only hope it will turn out that way. We still don’t know what caused this and may not every know. She says that the next couple of days we will have to wait and see how he does. We pray he continues in the direction that he is going. He is taking tiny baby steps, but it is the right direction.

Please continue to remember Noah and the other bone marrow kids that are presently in the PICU, Jose David, Alex, Aristophanis, Garhette. Please remember to pray for the other PBMTU and Krabbe kids too. Thanks.

Saturday, May 24, 2003 8:50 PM CDT

This is an update from last night. Noah had a bad and very scary night. His hemoglobin dropped down to 4, his heart rate was dropping fast into the 40s-50s, he was requiring maximum ventilation on the conventional ventilator and was still not maintaining adequate oxygen saturations. He required A LOT of blood to be pushed through his IV fast and some platelets to try and help stop bleeding. His chest x-ray showed that he likely had a hemorrhaging lung and blood was filling them, especially the right one. He was placed onto a "Last Chance" ventilator known as the oscillator and was at maximum settings. He was started on a broad range of antibiotics. Over the course of the day he has made some small improvements, but he remains still very, very ill and could still go either way very quickly. We continue to ask for your immediate prayers that they can find the source of this mess. They cannot explain why fluid was leaking into his abdomen and fluid & blood into his lungs. We will try to update tomorrow to let everyone know how tonight goes. Thanks.

Friday, May 23, 2003 9:19 PM CDT

Entry 102---May 23, 2003 Post Transplant Day 93/ Post Transplant Boost Day 30

Well, I am sure some of you are eagerly awaiting this journal entry, but I must first say a few things before giving the news that we have all been waiting for today. I must admit that I was pretty certain that Noah’s test results would return today at zero and that we would be stuck in the middle of a HUGE decision…to transplant again and accept the risks that go along with it, or to throw in the towel and take him home to allow him to rest until he finally died. I had pretty much run out of patience and hope…I am so very, very tired of waiting to see how things are going to pan out for us. I want so much for him to get well if that is his fate, or for us to go home if he not destined to get well, but I want it to happen REAL SOON!! Of course, if I knew that he was getting well, then I would be more likely to be more patient and willing to wait it out until he was at a point where we could take him home, but everyday of every week brings us something new and when we start to have something go a little bit in the right direction, we are shot even further back in the opposite direction. And I don’t think I can handle anymore of this back and forth stuff. My patience and emotions are running so very, very thin. So, since his donor cells are now at 10up from 3.50 days ago), I don’t think I could handle it very well if they went back down. Yes, I did say that Noah’s donor cells are now at 10! I have eaten my words and asked forgiveness for my lack of faith and hope. We will have to wait now a couple more weeks to see if there is either a rise or fall by testing him again…we are DESPERATELY praying, begging and pleading with God to work his powerful Heavenly ‘magic’ and place some “Heavenly Miracle Grow” on Noah’s Donor Cells. Is everyone doing the “DONOR” Cell Dance?? Well, please do it as often as you can…and pray a lot for them to grow (**please specify ‘donor’ cells**). We feel so blessed to have received this GREAT news today…THANK YOU GOD!!! And THANK YOU TO ALL OF YOU WHO HAVE BEEN PRAYING SO HARD FOR NOAH!! THANK YOU, THANK YOU, THANK YOU!!!! We have so far to go yet, but this brings us some hope. We do know how quickly his donor cells can be lost though and this is a little scary, but we will pray and hope for the best.
Noah is going to have his EVD drain internalized on Tuesday, May 27th, and he will have his MRI and other tests later next week. We are praying that this all goes well and that the surgery will not stunt his donor cell growth. He is hanging in there, staying so strong and courageous. We couldn’t be prouder. We are so very thankful to everyone for the prayers and support that you have offered. THANK YOU!! We continue to ask for your prayers and support for Noah, for Kendal and Natasha’s families who are grieving their passing, for the other PBMTU who are on the bone marrow unit and in the PICU, the other Krabbe kids and their families, and for Grammy Prichard’s safe return home on Sunday. She has been so great about staying with Noah during the nights so that dad and mom can actually get some sound sleep. We want to add another Thank you to The Kristoff family for dropping in to visit us and to see Noah. Thank you for the sweet care package. Their son Ryan was transplanted for Krabbe 18 months ago at Duke and returned for his check-up. They have offered us a lot of hope and tremendous peace about our decision. Thank you Pam and Bill. It was so very nice to meet you all. Thank you to Greg’s M.P. unit back in Hawaii for showing your support by allowing him the special arrangements to stay here with Noah during this rough time. Thank you to Kapi’olani’s PICU staff for the incredible support that you have shown. I got your message Helen…thanks for checking in on us. I cannot wait until we are back home and I can come back to work with you guys/gals. You are all so great. God bless you all. Thank you Kerri, Jason and Judy for the wonderful care package you sent us. That was so very kind and generous of you...we are so grateful!

Please continue to check in on our other Krabbe kid here at Duke, Elijah, who has begun to grow his cells. He is at 0.3 today!! He had a bone marrow aspiration today to check and see what his bone marrow is doing. So he had a rough day, but he has cells!! His web page is www.caringbridge.org/il/elijahbarron

Thursday, May 22, 2003 8:57 PM CDT

Entry 100--May 22, 2003 Post Transplant Day 92/ Post Transplant Boost Day 29

We know that all of you are waiting patiently for news of Noah’s lab results, but we did not get them today so we will have to wait and see what tomorrow brings. They are looking to internalize Noah’s shunt on Tuesday, his eighth surgery, and his MRI is scheduled for Thursday. So even if we get the results we will be missing an important peace of the puzzle, his MRI. You can’t really hold Noah because of his EVD but today I put the rail of his crib down and bent down and laid my head next to his and we had a talk, as we often do. I rubbed his back and told him how much I loved him, and that I whish he could tell us how he was feeling and what he wanted us to do. He was sound a sleep and I put my finger under his hand and he squeezed it, he has always loved to have his hand held and his grasp has always told us how he is doing. When he is hurting it is tight and when he is relaxed or sleeping it is lose. But today it was neither, not to tight and not to lose, firm as to say, “I know you’re there Dad and I love you.” I guess that’s the best answer I could ask for. Another family lost their son today on the floor, Kendal age 16, lost his fight with cancer, please pray for the grieving parents. Please continue to keep all the families, doctors, and children who fight so hard for life in your prayers.

Wednesday, May 21, 2003 11:20 PM CDT

Entry 100--May 22, 2003 Post Transplant Day 92/ Post Transplant Boost Day 29

We are waiting…waiting for Noah’s lab results. This is difficult because we know that the results will cause us to make a complicated decision. What would you do, if you had a child with a fatal illness and there was a remote chance to save him? After all the difficulties of the first transplant could you allow another one? Could you live with refusing a second transplant and a chance at life? I find it hard to look at my son and say, Okay enough is enough let him go. What if we took him home and as the effects of chemo wore off he started to get better? Would we not be tormented by the fact that we didn’t give him a chance? Would it torment you if you were his parents? I am scared to walk away from treatment because as he has shown us anything is possible. He could surprise everyone by swinging in the other direction, by deifying the odds in a positive way. If we op for another transplant then we realize it could be the end of him…but we also know if we don’t he will die. So…what to do? We will pray that God will help us with the answer.

Monday, May 19, 2003 1:11 AM CDT

Entry 99---May 19, 2003 Post Transplant Day 89/ Post Transplant Boost Day 27

There is a new change to Noah’s website at the request of the nursing staff. If you look just above this entry you will see NOAH SAYS, and then a short commentary. The story behind this is that in Noah’s room there is a dry erase board and every morning Dad puts a new Noah says for the day. It usually covers how he did over the last 24 hours or reflects problems and frustrations. Manny of the nurses will come by to see what it says and one of them suggested we should put it on his web site so they can check it when they are off. Not one to let down some of his biggest fans, we took her suggestion and have incorporated it into his site. We hope everyone enjoys the entries. Today Jeff Foxworthy and his wife Gregg visited Noah. They were both very nice, down to earth people who showed a genuine concern and interest in Noah’s condition. We would like to thank them for stopping by and brightening our day. Noah opened his eyes today and has been able to keep is O2 saturations up with little to no assistance. His feeds also started today through his new G-Tube and have been going well, so well he had a poo while I was writing this. Dr. K came by to see him again today and is pleased with how well he has recovered after a ruff couple of days. We asked her if she felt that Noah’s illness had progressed and she told us that most of what we were seeing was due to the chemo and that she did not see indications that it had. We are so glad she was on this weekend, she has really helped us sort through some of the symptoms we have seen Noah exhibiting, and helped give us a better understanding while calming some of our paranoia. All in all Noah had a good Sunday and we are thankful for that. We hope to see continued improvement as this week is going to be a long one while we wait for the results of his donor cell status. Thank you for the prayers and continued support through e-mail and journal entries, they mean so much to us. Please continue to include all the families and children in your prayers that suffer from these terrible illnesses,especially the family of Natasha who lost her fight with Krabbe’s Saturday night.

Saturday, May 17, 2003 9:47 PM CDT

Entry 98---May 17, 2003 Post Transplant Day 87/ Post Transplant Boost Day 25

This will be a brief update for those who check his web page on the weekends. Noah’s surgery went well yesterday, or so we thought. When we went back to the recovery room to see him, we noticed air in his EVD drain and asked about it. We were concerned and suggested that the recovery nurse contact the neurosurgery team. She had mentioned that she had clamped one of the clamps on the bag…and we aren’t sure why since they are not supposed to be open except only when emptying the chamber that the spinal fluid drips into after it is full, but is then quickly clamped again. This sent off huge buzzers and whistles that someone messed with his drain. When Noah was returned back to his room we mentioned to the bone marrow nurse what we had seen a couple of hours earlier…and that he still had not drained off any fluid…which is totally abnormal. A neurosurgery resident came in and suggested getting a CT scan of his head. Noah had his CT scan and we waited, and waited, and waited, and waited…finally at about 0400 this morning we learned that Noah had an increased amount of fluid building up in his brain and that he would need to have his EVD removed and another replaced in surgery. So Noah was rushed off to surgery again, a surgery that he should not have had but due to some person’s negligence, he was now at a threat and must be operated on. We were very upset that our 8-month-old son was being put into harms way of undergoing anesthesia and a type of brain surgery because of some adult’s negligence…not paying attention and being extremely careless & unsafe. Somewhere between the time we said goodbye to him in the pre-op room and he was rolled away by anesthesia, to the time that we were allowed to come back to see him in the post-op recovery, his EVD tubing had gotten a large amount of air in it and his drip chamber totally emptied. As well, the tubing that ‘was’ sutured down to his skin at the insertion site was over 2 inches out from the skin…which was another clue that it had been tugged on and pulled out. We voiced our deep frustration and concern about all of this and of course, all that we were told was…”We are so sorry this happened, it is very unfortunate, this is not “Duke Medicine”,…but it doesn’t change the fact that Noah has to suffer because of it! This is why we are so fearful of ever leaving his bedside, because we have to watch to make sure that nothing bad happens to him…and because we could not be in the O.R. with him…we could not watch what they were doing so it was out of our control. This is not the first time that something bad has happened because we were not around to protect him (as happened in the PICU a few times), but this is by far one of the worst things ever. We are running ourselves ragged trying to be up and with him all of the time to try and protect him. BUT…we know that we MUST be there or he will continue to get hurt. Noah is recovering in his room now and resting fairly well. His level on the EVD was reduced back down to 4, and he seems to be tolerating this much, much better than at 6. His heart rate has been relatively more stable without any major dips in it so far. We will see what tomorrow brings. Thanks for the continued prayers, Noah needs them!!

Friday, May 16, 2003 3:43 PM CDT

Entry 97---May 16, 2003 Post Transplant Day 86/ Post Transplant Boost Day 24

Today was surgery day, and the surgery itself went well. His liver is enlarged and the surgeon had to make a larger than normal incision to work around it, but everything went fine. His G-Tube for feeding is in place, but he won’t start feeds for another day or so. He was successfully extubated and was able to spend some time in the post-op recovery to get his pain under control, and then return to his room on the PBMTU! Praise God for that! We are so relieved that he did so well and did not have to go to the PICU. He is in A LOT OF SEVERE PAIN though! He required a lot of narcotic pain medication, but still seems to have some underlying discomfort. Hopefully this will soon subside and he will quickly heal and begin to feel better in a day or so. We will be praying hard that he heals quickly, suffering very little from any pain, that he does not develop any infection, and that his belly begins tolerating his tube feedings again. We would appreciate your prayers on these matters for Noah’s sake. It is so nice to see his beautiful face with those cute chubby cheeks without that yucky tube taped to his face. But when one is removed in one place, another is replaced somewhere else, so he has a new coming out of his belly. That is the way it goes. We appreciate all of the wonderful prayers for Noah and his surgery…prayers were answered as he did well. Thank you to those of you who have visited Elijah’s web page and offered him and his mom support. His site is www.caringbridge.org/il/elijahbarron His mom Carolyn has a daughter, Alissa, who also has Krabbe. She needs a lot of support and encouragement during this time. Elijah still awaits his cells to grow and he is already over 30 days post transplant. We ask that you remember them in your prayers. We also would like to ask that you pray for Brent, who has been here a little longer than Noah and has not made it home yet. His web page is www.caringbridge.org/ga/brentr Please continue your prayers for all of the PBMTU kids (and Alex & Kendal who are in PICU), Krabbe kids and their families. God bless.

Wednesday, May 14, 2003 5:50 PM CDT

Entry 96---May 14, 2003 Post Transplant Day 84/ Post Transplant Boost Day 22

We missed writing a journal yesterday, shame on us for being lazy! ;-) Noah had an upper G.I. tract series done to check the anatomy to make sure it was normal…and it is. They also pulled his feeding tube out a bit so that it was pulled out of the top part of his intestines back into his stomach. Then they put a small amount of formula into his stomach and waited an hour to see how much would empty into his bowels out of his stomach within that time. He did well on this so it won’t require any intervention. He did not show any signs of gastric reflux during the procedure, but they feel that he may develop this problem in the future and want to go ahead and plan to do a procedure called a ‘Nissen Fundoplication’, where part of the stomach is pulled around the top sphincter of the stomach and stitched into place, to prevent stomach contents from going back up into the esophagus. Then they want to place a permanent feeding tube through his abdomen into his stomach. This will allow them to take out the one that goes through his nose and down his esophagus into his stomach, and will also allow us to feed him a few ounces at one time and then disconnect him from the pump…preventing him from being tied to a pump all of the time. This will be especially helpful when we take him home. So the question is when is all of this happening? Friday, in two days Noah will go to surgery. We are not certain of the time, but we pray that everything goes smoothly and that he is able to come out of surgery without any problems. Our main concern is that he won’t be able to breathe on his own after surgery and will require going to the PICU to remain on a ventilator until he will wake up and breathe on his own. So we are definitely praying hard that he does well throughout this. After this surgery, he should only have one more left and that one will be for the placement of his internal shunt. We continue to pray that things move forward so that we can take him home soon. Tomorrow he will likely get platelets and some other pre-op interventions to prepare him for Friday.

We owe many thanks to so many people, and it would be irresponsible to try and name everyone, but we want to mention some that come quickly to our minds at the moment. Thank you to Grammy Prichard for staying with Noah for the last few nights and doing such a great job of loving on Noah. Thank you to Jamie, Jeanne, Grammy P., Mom & Nat, Greg & Noah, and all of the others for the wonderful Mother’s Day gifts. Thank you so much for making it such a special day, which I will always remember. Thank you for all of the prayers and support for Noah and us during this difficult time. Thank you to everyone at Grammy P’s work in Fresno for allowing her the extra time to be here for Noah. God’s blessings to everyone for the extreme kindness you have shown to us during this time. We continue to ask for your prayers for Noah, the other Krabbe kids, PBMTU kids/families, and those Krabbe families making their way to New York for the Hunter’s Hope Symposium 2003. We wish we could have been there, but maybe next year. We ask that you will visit Elijah Barron’s web page, another Krabbe kid here on the bone marrow unit. He is waiting for his cells to grow, and needs your support. His web page is www.caringbridge.org/il/elijahbarron

Monday, May 12, 2003 10:42 PM CDT

Entry 95---May 12, 2003 Post Transplant Day 82/ Post Transplant Boost Day 20

This entry will be a brief run down of Noah’s latest status. Noah is fairly stable since they have made no real changes this weekend. His EVD drain pressure limit remains at 6, and he seems to be adjusting to it slowly, having infrequent drops in heart rate. His tube-feeding rate is up to 23cc/hour (4 ½ teaspoons/hour). The goal is to reach at least 30 cc/hour, so we are getting close and that is nice to see. The plan of care has changed a bit from last week. Before the goal was to try and get the shunt internalized ASAP, but if they do that and then later try to put in a permanent gastric feeding tube via abdominal surgery, then we again run the risk of having his belly pressure increasing from swelling post surgery and thus causing his shunt not to drain effectively as was the problem before…leading to the shunt being removed and the external drain being placed. So, why would we want to risk having to put him through any more surgeries than necessary, well, we don’t. So the plan is to do the gastric feeding tube placed first, leaving the external drain in place for now until he no longer has any abdominal swelling and his tube feedings are started up again. Then we can try to have them internalize the shunt after his belly is up and running again so that we don’t have any pressure issues. He must be checked out by the GI docs and the pediatric surgery team this week, have a few diagnostic tests done, and then design a time schedule as to when this will all be done. It will most likely be done next week. Other than that he is relatively stable. His Grammy Prichard has been staying the night with him for the last two nights and tonight too. We say THANK YOU to her for giving the chance to get a break and some much-needed sleep. Thanks to everyone for all of the wonderful words of support and wishes for Mother’s Day. We ask that everyone will continue to keep Noah in your prayers, as well as the other PBMTU kids, especially Kendal who is in the PICU, and the other Krabbe kids. God bless.

Sunday, May 11, 2003 2:19 PM CDT

Entry 94---May 11, 2003 Post Transplant Day 81/ Post Transplant Boost Day 19

Mother’s day had never been a holiday that ever meant much to me. Don’t get me wrong, Mom’s deserve to be honored on Mother’s day after all, they are the ones that bore us into this world, nurtured us, provided for us, and taught us some of life’s most important lessons, lessons Dad’s have just never been so good at teaching. So I realize the importance of the day but it never rated up there in the top three like Christmas, Easter and Thanksgiving. This Mother’s day is different, it has a special significance due to Noah’s condition and because it’s Debbie’s first. It has been hard on both of us, but especially difficult on Debbie. You always hear about the special bond between mother and child but those feelings can’t be easily described or recorded on paper. I was able to experience the power of that bond on two occasions, the first, when Noah was diagnosed and the second, when we were told his in transplant was failing. I stood there dumbfounded when they told us, watching in utter shock as Debbie picked her son up, clutching him tightly to her chest and cried out and wailed “My baby, my baby.” The experience passed right through me to my soul and will forever be seared into my memory. It overwhelmed me and made my knees weak to witness these moments, and I knew that I had been given an intimate glimpse of Debbie’s love for her son. I will never forget those moments because I saw her soul for a brief moment, her body and brain unable to control or contain her love for Noah as it burst from within and filled the room, leaving me in awe. And so on Mother’s day I am taken back to those moments and I think with every card or present Debbie is too. We will never forget this Mother’s day; it will remain in our recollections forever. Mother’s day will evermore have a new significance in our family because of the memories it now carries, and the tears it has brought.

Saturday, May 10, 2003 4:39 PM CDT

This truly has been the most blessed and memorable mothers day in my lifetime. My daughter in her motherhood and my daughter in law in hers have showed me a fulfillment of mothering. What beautiful selfless loving women they are totally dedicated to their families. God deserves the glory in all out lives but I’ll be eternally grateful that he touched us so beautifully. As some of you know I’ve had my hours of darkness but spending time with this precious angel and my wonderful kids has restored my spirit. It’s all about Noah! This child has impacted his family and all humanity more than he’ll ever know. Kathy once said he is a very special angel from God and I can see now what she meant and then some. Beside which he’s way cute! I pray our hearts remain open as God continues to carry us through His plan. God bless and happy Mothers Day.
Noah’s Grandma Prichard

Saturday, May 10, 2003 0:52 AM CDT

Entry 93---May 10, 2003 Post Transplant Day 80/ Post Transplant Boost Day 18

This Mother’s Day will be a little bittersweet for me. Although it is not been confirmed that Noah is not engrafted, there is that inner voice, (mother’s intuition??), that tells me that Noah will not be engrafting and we will be taking him home to die from his Krabbe Disease after his other immediate issues are fixed over the next few weeks. This is not to say that I don’t have faith in God and what he can do, or that I don’t believe in miracles. I do believe in miracles. I believe that Greg and I miraculously met on a blind date and fell in love immediately over 7 years ago…a love that remains ever so very strong and is only strengthened by this experience. I believe that it is a miracle that we were blessed with the sweet little boy that we wanted so much, and conceived almost as soon as we mentioned that we wanted to start a family. I believe it is a miracle that we discovered that Noah could have a transplant for his terminal disease and we were able to get here to Duke within 6 days of learning that there was something seriously wrong with our baby. I believe that it is a miracle that we have found the strength within to endure this horrendous transplant & Krabbe experience…and that it just keeps coming with each new hurdle that we have had to face. I have been able to see such small miracles that many take for granted. I have seen my baby smile and I have a photo to keep forever of his beautiful smile. I have heard the sound of his little laugh and seen the wonder in his eyes of discovery. I have witnessed his ability to hold his head up, move his arms and legs, and roll from his back to his side. I have witnessed a miracle at his ability to maintain eye contact with me and tolerate sitting up for short periods of time in his special chair. I have witnessed a small miracle at being able to stick my finger in his hand and have him grab onto it tightly, and watching him calm at the sound of my voice or touch of my hand. I do believe in miracles and have certainly seen them. I must say that it saddens me that I have not seen a smile, heard a laugh, watched Noah move & roll anymore, but to have seen them once and have them forever imprinted on my heart and mind is a blessing. As well, there are many things that I have not been yet blessed to see with Noah, such as sitting up without support, crawling, walking, babbling and talking, playing with toys or riding a bike, but I am so very grateful for the small miracles that I have witnessed.
And faith?…I have faith that God was responsible for all of these little miracles in our lives and have faith that he will carry us through this experience. I have faith that one day, when I am in Heaven, that I will understand why things like this happen and why we were chosen to undergo this difficult journey. I cannot deny that I question “Why me?” or “Why my baby?”. I cannot deny that I have been angry and hurt by God’s choosing me to have to endure the high probability of losing my firstborn to a fatal disease. I am not alone, Greg hurts just as much, and we are blessed to have each other to turn to throughout all of this. We understand the pain that each other is enduring and it is a blessing not to be alone. At times, one is more at peace with it all than the other, and then on other days, we exchange our positions and the one that was at peace begins getting angry all over again. We can only pray and hope that we have been the absolute best parents to Noah that we could possibly be, show him as much love as we possibly can while he is here with us, and honor him by sharing about him and his disease with as many people who will listen. I really liked the phrase that someone placed on Noah’s guest book a few days ago, “Sunshine or Rain, God can use both to help us grow”, or something to that affect. We feel like we have been slammed by a gigantic tsunami (tidal wave) these last 7 ½ months and have not seen sunshine for some time. But, we are still growing nonetheless. I must let everyone know that it was Greg that wrote the inspiring entry on Friday. I think he found a lot of peace from Pastor’s visit the other day and felt compelled to share what he brought from it. He is a very talented author & artist, and is able to express his thoughts and emotions in such creative, poetic form that touches your heart and provokes thought every time you read it…he never ceases to amaze me with his talents. Whereas I am more matter of fact and simple, but that is why we get along so well. His journals certainly tug at my heartstrings, and I am so pleased that he is able to touch so many of you as well. In celebration of Mother’s Day I decided to write a bit and then I wanted to post a brief story that was sent to me by another mom with a child who has a fatal disease. I hope that the moms out there all have a wonderful Mother’s Day. I want to wish my mom, sister, step mom, and mom-in-law, & sisters-in law all a very Happy Mom’s Day. Thank you for the wonderful support from everyone these last couple of weeks in particular. We ask that you will continue to pray for Noah, the PBMTU kids, Krabbe kids, and special requests for Natasha, Maddie, and Makayla Pike’s family. God bless.

GOD TAKES CARE IN PICKING MOMS FOR "SPECIAL" KIDS
Most women become mothers by accident, some by choice, a few by social pressures and a couple by habit. This year, nearly 100,000 women will become mothers of handicapped children. Did you ever wonder how mothers of handicapped children are chosen??? Somehow I visualize God hovering over earth selecting His instruments for propagation with great care and deliberation. As He observes, He instructs His angels to make notes in a giant ledger. God passes a name to the angel and smiles, "Give her a handicapped child." The angel is curious, "Why this one God? She's so happy?" "Exactly," smiles God. "Could I give a handicapped child to a mother who does not know laughter? That would be cruel." "But has she patience?" asks the angel. "I don't want her to have too much patience or she will drown in a sea of self-pity and despair. Once the shock and resentment wears off, she'll handle it. I watched her today. She has that feeling of self and independence that is so rare and so necessary in a mother. You see, the child I'm going to give her has his own world. She has to make him live in her world and that's not going to be easy." "But, Lord, I don't think she even believes in you." God smiles. "No matter. I can fix that. This one is perfect. She has just enough selfishness." The angel gasps, "Selfishness? Is that a virtue?" God nods. "If she can't separate herself from the child occasionally she'll never survive. Yes, here is a woman whom I will bless with a child less than perfect. She doesn't realize it yet, but she is to be envied. She will never consider a step ordinary. When her child says 'Momma' for the first time she will be present at a miracle and know it! When she describes a tree or a sunset to her blind child, she will see it as few people ever see my creations." "I will allow her to see clearly the things I see . . . ignorance, cruelty, prejudice . . . and allow her to rise above them. She will never be alone. I will be at her side every minute of every day of her life because she is doing my work as surely as she is here by my side." "And what about a patron saint?" asks the angel, his pen poised in mid-air. God smiles. "A mirror will suffice."
-Author unknown

Thursday, May 8, 2003 2:00 PM CDT

Entry 92---May 8, 2003 Post Transplant Day 78/ Post Transplant Boost Day 16

Yesterday a Pastor from a local Lutheran church came to see us. We prayed over Noah, experienced God’s love and grace through the Sacrament of Communion, and had Noah blessed. During our discussion and prayer with Pastor something extremely obvious came to me and hit me like a ton of bricks. Often people will tell you that they can’t know how you feel, and they are right. They may have had a similar experience, but our feelings on this matter are uniquely ours. You cannot simulate in your imagination the deep bond there is between our son and us; we are close in a way no journal entry could ever capture. And for that reason, since you don’t know our hearts, you can never know what we are going through, even if you have lost a child or been through the transplant process. There is only one person who can tell me they know what I’m going through, and that is because they lost their son to a terrible death and they also know my heart. That person is God. I knew that all along but for some reason my tears had clouded my vision so badly I couldn’t see it. None of this changes Noah’s clinical outlook but it helps to remind me that God weeps with us at the crib side, because he intimately knows the pain we feel. Don’t get me wrong, I still don’t understand, know, or like, the hows or whys of this tragedy. What I am able to do more now is lean on God. He is back like an old friend you decided to look up during a difficult time in your life, a friend who never left you but who you… failed to keep in touch with. In the end His will be done, and through the love of Christ our family will be together one day with Him.

Wednesday, May 7, 2003 11:24 AM CDT

Entry 91---May 7, 2003 Post Transplant Day 77/ Post Transplant Boost Day 15

Debbie has been doing a great job keeping everyone up to date on Noah’s status. If you look at Noah’s progress over the last few weeks from a clinical point of view, it doesn’t look that bad. It appears that he is making some headway with the feeds and should soon be able to tolerate the placing of an internal shunt. I don’t see him the same way; the lab results, O2 saturations, heart rates and other tests are numbers to me. I realize they are important but I don’t always understand them and I am not convinced they portray an accurate picture of Noah’s condition. I look more at how he responds to me. I look into his eyes, they have always told me so much. I hold his hand, or rub his back, whisper in his ear, or kiss his checks, and watch for a response, outwardly and on the monitors. That tells me so much more. Debbie has always been quick to remind the doctors here the golden rule of, “look at the patient” not the CT scan or the labs. The patient will often give you a better picture of what is going on. So what is the point that I am trying to make here? The point is that the journal entries that Debbie does are clinically accurate; they reflect his lab results and her clinical observations for the day. As you read them it appears on paper, that his condition is getting better. If you were to see him like we do, day in and day out though, you would probably begin to come to the conclusion that he is not doing very well at all. On the rare occasions that Noah opens his eyes now he is not there, he looks right through you, he can’t see you. He is slowly slipping away; unable to keep the oxygen levels in his blood up without blow by oxygen and his airway clear without constant suction. We use to write this off to the transplant process, confident that it will pass, but it hasn’t… it only seems he is needing more not less assistance. So what is causing this? We believe it is the progression of his illness…his central nervous system is not controlling his body like it should, and this doesn’t show up on labs. Next question is probably how can that be if he had a normal enzyme level? Unfortunately, he had that level over a month ago, and they have not tested for it since. With the loss of donor cells the enzyme has also gone. It is doubtful he had the enzyme long enough to do any real good since it usually takes months to even get to the brain. So, what about the boost you ask? Well, we see no evidence that it is working, no rash, no fever, nothing. We know what to look for because we spent a month looking for it after his first transplant and it’s not there. We have seen the spikes in his white blood cell counts, which indicate an immune response, but we realize it is his old blood system responding to the boost and probably killing it as an invader. It would be nice to hear good news later this month, that his donor cells are back and his enzyme level is normal but… that is just wishful thinking. If you looked at Noah you would know that is not going to happen. We have been forced to open our eyes to what we see and listen to what our hearts tell us. If there is one thing this experience has taught us it’s that parent’s intuition has always been better than any labs. Our hearts are breaking now, we are tired and depressed, and we can only hope that God will make His will known soon. We had hoped we would never be that family sobbing in the corner, that you feel for but don’t quite know what to say to them. After all what can you say? So you pat them on the shoulder, hand them a tissue, and leave. Unfortunately we have become that family the only question is… for how long.

Tuesday, May 6, 2003 5:56 PM CDT

Entry 90---May 6, 2003 Post Transplant Day 76/ Post Transplant Boost Day 14

HAPPY NURSES DAY!! In celebration of Nurses’ Day/ Nurses’ Week, we want to offer a huge THANK YOU for all of the great care you have provided us and Noah during this process. Jeanne, we could not have asked for a better Primary! Thanks for all of the bedside “Coffee Talks”.

Today Noah’s drain pressure limit was increased to 6, per ordered by the neurosurgeon. He has been having relatively frequent heart rate drops and desaturations all throughout the day. We are a little alarmed, but maybe he will be able to adjust to this raise in pressure limit over the next few days. We have just been keeping a close eye on him all day. It looks like it will be a long night. His tube feeding was increased to 15cc/hour (3 teaspoons/hour), and so far he is tolerating it well. We received word about the date that his next chimera blood test will be done to count the number of donor cells. His next big lab day is on Tuesday, May 20th. This will be a big day for us all. We continue to pray for engraftment to happen…soon, and for his GI and shunt issues to resolve themselves in the meantime. We are signing up to have another virtual quilt made for Noah at www.quiltsoflove.com . It won’t be ready for another week or so, but once it is we will add it to the other quilt site up above. Thank you for all of the prayers.

Monday, May 5, 2003 9:34 PM CDT

Entry 89---May 5, 2003 Post Transplant Day 75/ Post Transplant Boost Day 13

Another pretty good day, Noah’s heart rate has stabilized a bit more since having the pressure limit lowered back to 5. We happen to run into the neurosurgery attending doctor and asked him about Noah. He said that if Noah absolutely would not tolerate anything higher than 5, then we would have to try and work with it, but that he would like to have some “room to play”. Normal belly pressure is around 3-5, so it would be all right if the shunt was left at 5, because it would still drain into Noah’s abdomen just fine, as long as his belly pressure remained within a normal range. However, if Noah’s belly pressure increased like it had done previously, preventing the shunt from draining any spinal fluid, then Noah would again have increased pressure build-up in his brain…and we would have the same problem as last time. So the neurosurgeon would like Noah to be able to tolerate pressure limits on the shunt of at least 7-10 if possible, just in case if Noah’s belly pressure ever did rise again, he would have less chance of having problems tolerating it. Therefore, as much as we can tell, their overall goal before Noah gets his shunt placed is to see if he can eventually tolerate increasing the drain pressure limit as high as possible. This will have to be done very slowly though, and may hinder placement of the internal shunt a couple more weeks. This might be just as well though, so that we can continue to gradually increase the rate of his tube feeding and get his abdomen moving even more. Noah’s rate was increased to 13 cc/hour today (2 ½ teaspoons/hour), and he seems to be all right with it thus far. The nurses measure his belly girth each day to make sure that it is not increasing too much. As long as it doesn’t get swollen and bloated, then it is a good indication that he is tolerating and digesting the feeds (along with him having poopy diapers). As for the cells, well, we need DONOR cells!!! We are desperately praying for donor cells to grow!! We need lots and lots of prayer for donor cells! We praise God for the answered prayer of getting his belly moving and working again, but in the end it won’t matter if he does not engraft with donor cells, because he will then die from the Krabbe. We are pleading for prayers for his donor cells to grow. Thank you to everyone for the wonderful prayers. Some of our prayers have definitely been answered and we are truly blessed, but Noah needs prayer for so many things. We pray that his donor cells grow…grow faster than we can say “GROW DONOR CELLS GROW”…grow so strong, allowing him to make the enzyme that will stop his Krabbe disease and save his life. We pray desperately for God to heal his frail, weak, sick little body. We continue to pray for all of the Krabbe and PBMTU kids. We offer a big “Congratulations” to Jeremiah, who made his jailbreak today from the bone marrow unit to start his outpatient journey. Please visit his web page at www.caringbridge.org/ia/jeremiah to offer him support and encouragement. We want to Thank Dave Forslind for stopping in to meet Noah and for wishing us well. It was so nice to meet you, and we hope that your week here with Kyle is going well. Hopefully we will be able to meet Fran and Kyle before you leave, but if not, please have a safe trip back home. God bless.

Sunday, May 4, 2003 8:13 PM CDT

Entry 88---May 4, 2003 Post Transplant Day 74/ Post Transplant Boost Day 12

Today we grieve yet another sweet child that has lost her life to Krabbe. Makayla Pike, just turned two last month, died early this morning at home with her family. She was not transplanted, but just the same had a long treacherous journey fighting her disease. We ask that prayers be lifted up in honor of this family and sweet Makayla during this very sad time. They have a caring bridge site for her and we ask that you visit it and offer them support at www.caringbridge.org/mn/makaylapike Their family has been very supportive to us throughout Noah’s transplant journey. God bless them. This has been such a hard week for us since having to bear the loss of Travis Williams, Ryan Davis, and Makayla Pike. We ask that you continue to lift all of these families up in prayer.

Noah’s external drain pressure limit was increased on Friday from 3 up to 5. This means that any excess fluid that is over the pressure level of 5, will drain off. However, this also means that he must be able to endure a higher pressure of 5 now, and Noah is very sensitive to Intracranial Pressure (ICP) changes. This was seen when he had his first internal shunt placed and two days later he began having heart rate drops and oxygen desaturations, which was because it was not draining off the excess fluid properly and the pressure was building up. The plan that one of the neurosurgery residents shared with the bone marrow docs was that Noah should be able to tolerate higher ICP’s without having any heart rate issues, just in case his belly pressure is on the higher side sometimes So this means that they want to increase his pressure limit on his external drain before it is internalized, so that was why they increased the limit on Friday from 3 to 5. Since everyone (except us) seems to be pushing to hurry up and get the shunt internalized, they now are trying to speed up the tube feedings each day, and neurosurgery is trying to hurriedly increase the pressure limit. Early this morning one of the neurosurgery residents came by and wrote an order to have the pressure limit increased to 7.5 from 5. A few hours later he began the heart rate drops again and was sleepier than normal. Our first thought, “Here we go again”, we just get him stabilized and tolerating his tube feedings and they go and mess with his ICP, which will again mess him up and eventually cause him to have heart rate drops, blood pressure increases, oxygen desaturations, irregular respiratory pattern, lethargy, vomiting, etc. We will be back to where we were a month and a half ago, because his tube feedings would have to be stopped once he begins vomiting. We thought for sure that we were once again going to be spending the next few days arguing and going to battle once again to get someone to listen to us that Noah is not tolerating the increased pressure limit on his drain. Fortunately, the attending doc today was one of the ones that was here during part of the previous time that Noah had so many issues due to the increased ICP. With a little pushing from Noah’s primary nurse, Jeanne, the doc came in and agreed that Noah was not tolerating the change at all, that it was too much for him too fast. He ordered to turn the pressure limit back down to 5 for the time until he could speak with the neurosurgery-attending doctor. He came back later and told us that the neurosurgeon said that normal belly pressure is between 3-5, and that it would be alright if Noah stayed at 5 on his drain. However, then we would need to definitely make sure that his gut was moving and doing really well before taking him back to surgery. So we increased his tube feedings to 11cc/hour (just over 2 teaspoons/hour), which means that he is at around 9 oz. a day! Yeah Noah! He is still having some heart rate drops down into the upper 80s to low 90s, but returns to his normal rate around 120s. We will just have to monitor him closely throughout the night and the next couple of days. He is having plenty of poops, thanks for the poop prayers! ;-) (And to Grace, Greg says to make sure and light the candles for poo poo during the day, because Noah has been too busy at night ;-) We are so blessed that he had such a good week last week and that he has tolerated his tube feedings so well. We thank God for that. There is still so much that must be done until he is recovered enough to be released, but we are heading somewhat in the right direction.

As for his donor cells, that is still up in the air so to speak, and we have to wait a couple more weeks for the next test to be done. Until then we will just try to focus on his feedings and his shunt issue. We ask that you will continue to remember to pray for Noah’s donor cells to grow, grow, and grow! Thanks for all of the great prayers and support. It was so nice to see a lot of you out at the Rainbow Walk of Heroes yesterday, what a beautiful event it is. The balloon release made my heart weep with both joy and sadness, for those who have been successfully transplanted, and those who have successfully earned their wings. What a truly spiritual moment it was, but it left me feeling a little worried about where we would fit amongst the crowd of transplant families when all is said and done. Would we be the ones there releasing a balloon, watching it reach up to the heights of the heavens to where our angel baby awaits our reunion, or would we be there in celebration of our son’s second chance at life and a bright, hopeful future together as a family unit. Anyone have a crystal ball we can borrow? But if it doesn’t end the way we want, would we really want to know before hand? Probably not. Until we know, all we can do is pray. Pray, pray, cry and pray some more.

Saturday, May 3, 2003 7:43 PM CDT

Entry 87---May 3, 2003 Post Transplant Day 73/ Post Transplant Boost Day 11

Last night after a few hours of sleep, I finally gave in and got up knowing it was a lost cause. I followed my usual routine, turned on the radio, showered, and then caught the elevator down to the lobby. I greeted the morning crew at the desk and seeing it was a nice day decided to walk the long way, outside and around the parking garage. As I approached the semi-circle driveway of the hospital I noticed a lady briskly walking my direction followed by a little boy. He couldn’t have been any older then four and was obviously having problems keeping up with the pace his mother was setting. His hands were outstretched and tears ran from his eyes all the time calling out for his mother. She finally stopped and in a harsh voice told him to “Shut up” and that he better, “hurry up” or she would leave him. Her pause gave him the chance to finally catch up to her and grab her hand, he stopped crying and in a jerky motion, his mother began walking again, holding his little hand tightly. I looked over my shoulder as she whisked him away towards the busy crosswalk. I have seen this before, parents trying to rush their children along, and it never caused me a second glance. After all, don’t we all have days when we are running late and become flustered? The only problem is that now with Noah, nothing looks quite the same. What I normally would have just written off as a rushing mother made me mad. I wanted to grab her by the arm, and hurriedly rush her up to Noah’s room and say, look at my son, he will never be able to run behind me, he will be lucky if he even sees his fourth birthday, don’t you realize what a wonderful gift you have? I appreciate the little things now like him opening his eyes to look at me, or when he holds my finger. O2 saturation levels above 93% without blow by oxygen or the ability to tolerate 10cc an hour of tube feedings devoid of throwing up are milestones to celebrate for our family. Babbling, smiling, rolling over, or even turning his head would be miracles for us, a reason to be thankful. It is amazing how tragedy opens your eyes to the world around you, and makes you appreciate the little things you have taken for granted for so long. Noah has been a great teacher and one thing is for sure the world will never look the same.

Friday, May 2, 2003 7:58 PM CDT

Entry 86---May 2, 2003 Post Transplant Day 72/ Post Transplant Boost Day 10

09:30 a.m.

Noah had a good night and slept like a baby! Yeah Noah! Well today we will likely learn of his test results that will show the percent of donor cells vs. his own bad cells. We are quite nervous about it. These next couple of weeks will be a defining moment for us all. If he is not engrafting then there most likely is not much more they will be able to do. They have not shared with us what, if anything, they will do if he does not engraft, but we are coming to the conclusion that there will not be really anything left. However, if he is starting to show a growth in donor cells then they will check again next week to make sure the trend continues in an upward direction. This is of course what we are hoping for. We will have to try and be patient and wait to see what the day brings.

20:30 p.m.

We received the news of his test results a few hours ago…and we are not hopeful, and extremely tired of having our emotions jerked around. Noah has had such a good week up to now, and he outwardly looks a lot better than last week, but his test results would say otherwise. He was at 23% donor cell before receiving his 3 days of ATG and his transplant boost of stem cells 10 days ago. His blood for this test was drawn 4 days ago and it shows that he is now at 3.5% donor cells. This is an incredible drop, and quite discouraging to say the least. We received an e-mail from Dr. K stating that she feels this blood work was drawn too early and that we will need to wait about another 2 weeks to take another blood sample to retest. Until then we will have to try and be patient, hopeful and work on the other issues that he has…his GI tract and his brain shunt. Gee, this is going to be hard, but again we are left without any other choices. There really is not much else to say. We are out of words, exhausted, and out of hope. We thank you for the prayers and words expressing your concern.

Thursday, May 1, 2003 1:20 PM CDT

Entry 85---May 1, 2003 Post Transplant Day 71/ Post Transplant Boost Day 9

Happy May Day! Today the PBMTU celebrated the Rainbow of Heroes Walk festivities for the inpatients & their families on the unit, since we won’t be able to participate in the actual event on Saturday. The staff passed out “Rainbow of Heroes” T-shirts to each patient room for the child and parent(s). They look real nice. On the back of them is the name of each child transplanted on the PBMTU from April 02 to March 03, so Noah’s name was included…along with some of the other Krabbe kids that were transplanted here within the year. The funds raised are donated to the PBMTU family support program to pay for art/craft supplies, weekly pizza for Wednesday lunch, and many, many other things that we can’t think of right now, but it is for a great cause. We donated $25 in Noah’s name and Kelly from Hunter’s Hope is going to participate in the walk on Saturday and represent all of the transplanted Krabbe kids. Thank you Kelly for representing our kids, and Thank you so much for the goodie bag! The banana bread was great! Noah has tolerated sitting up in his chair for a few hours yesterday and today. Although he sleeps most all of the time that he is sitting up because he is sedated, it is great to see that he tolerates it. His tube feeding was changed over to complete formula, without any Pedialyte today. He remains at 7cc/hour (about 1 ½ teaspoon/hour) and he is tolerating it fine so far. However, it might take a day or two to really determine if will tolerate the full strength formula. There are really no other changes to report. Noah’s Grammy Prichard is coming all the way from California to see him next week. We pray that she has a safe and speedy journey. We cannot say enough how delighted we are to report better news this week. Thank you for all of the incredible prayers. We say prayers of Thanks for the blessings that God has given. We ask that you will continue to remember him in prayer, along with the other PBMTU kids, especially Juan in the PICU, & the Krabbe kids.

Wednesday, April 30, 2003 1:19 AM CDT

Entry 84---April 30, 2003 Post Transplant Day 70/ Post Transplant Boost Day 8

What another sad day for us yesterday. Yesterday afternoon Travis passed away in the PICU. We offer our sincerest condolences to his family for their loss and pray for them as they endure this extremely difficult time of grief and sorrow. Please feel free to offer them support by visiting his web page at www.caringbridge.org/hi/twilliams You don’t have to be a family member, friend or acquaintance to offer support and encouragement...and we know personally just how meaningful the guest book messages are when having to endure the most difficult times of this long journey. We appreciate reading them daily, and often look on his web page a few times a day to read them. So please remember them especially in your prayers. As for Noah’s day, he had another pretty good day. His tube-feeding rate was increased slightly to 6cc/hour and so far he has been tolerating it well, except that he has not passed anything through his bowels yet. So again we wait for poop. We pray for poop! How funny. However, this will mean that he is digesting the feeds well and that his gut is starting to work. He was again started on the low dose antibiotic of Erythromycin, which also helps increase bowel motility as a side effect, and this will hopefully help him out with getting his belly moving. His chest x-ray done yesterday looks remarkably better than last weeks x-ray. He had his blood drawn for the percentage donor test and we will likely know the first of next week what it shows. We still don’t know what kind of “bug” may have been in his donor stem cell boost that he received last week, but hopefully will find out this week for sure if there is indeed a “bug” that is a threat to him. He has been a lot more awake than in the last month and it is great to see. He is still quite irritable at times when trying to reposition or hold him for short periods of time, but it is nice to see his eyes open. There really isn’t much else to tell as far as his progress or any changes, because he has remarkably been so stable the last few days. He hasn’t had too many times when he has remained so constant and it is a little scary. We have always had to be on our toes with Noah, and it is weird being able to sit back and relax a little and just wait to see what happens next. It seemed for so long that in the midst of trying to deal with one thing happening, we were already being bombarded with another catastrophe. We continue to pray that Noah has finally made a turn in the right direction, toward healing and recovery. We continue to pray for donor cells to grow and for him to engraft with his new donor cells. We wish we could speed things up a little faster, time seems to be going fast, but his recovery has been at a stand still for so long and is now only at barely a snail’s pace. We cannot complain though, he is doing better these last few days than he has been in a few weeks. Please keep him in your thoughts and prayers, along with the other kids, especially Juan who remains in the PICU still very sick. As for some good news, Nolan is back on the bone marrow unit after being in the PICU for a couple of weeks. Welcome back Nolan. You can check out his web page at www.caringbridge.org/co/nolanramsey to learn about his progress. Thanks and God bless.

Tuesday, April 29, 2003 1:23 AM CDT

Entry 83---April 29, 2003 Post Transplant Day 69/ Post Transplant Boost Day 7

Well, here is another early morning journal entry to share with you about yesterday’s events. Noah’s tube feedings were changed a bit today. His Pedialyte was mixed in half with formula. So far he has tolerated it without getting sick or having discomfort. He has quite a bit of discomfort when passing a stool since he has been out of practice for some time, but hopefully as his motility in his belly picks up the pace, he won’t have as much discomfort. There was not as much noise coming from his abdomen today, but it is moving a little and that is certainly better than not at all. So we’ll take it! That was the only major change for yesterday. Noah was awake a little more often, which we expect to see since his sedation meds were decreased last week. We praise God for the small improvements that Noah has made this weekend and pray that improvement and healing continues. We pray that Noah’s donor cells take over his body and outgrow his old bad cells. Thank you for all of the prayers and support him and us. You all have been great. God bless you. Thank you for praying for all of the other PBMTU, PICU, & Krabbe kids, and especially for Ryan’s family during their time of immense grief and sadness over his loss. We ask that you will continue to remember all of these kids and families in your thoughts and prayers. On a lighter and happier note, one of the PBMTU kids whose name is Harmen, made his jailbreak today and is now in the outpatient phase of the transplant journey. We wish him and his family the best and pray that he remains strong and well, never having to come back onto this unit except as a visitor.

Monday, April 28, 2003 0:35 AM CDT

Entry 82---April 28, 2003 Post Transplant Day 68/ Post Transplant Boost Day 6

It is so early in the morning on Monday, but since we have not made it to bed yet and did not manage to write a journal entry this weekend, we decided to write a brief update. Noah has been really stable this weekend, tolerating his Pedialyte tube feedings, which were increased up to a total of 5cc/hour (1 teaspoon/hour). He has been having some small stools every other day or so, which means that something must be moving in his belly if something is making its way out. We never thought we would ever be so excited to see stool! Yeah Noah!! Yesterday there was a few gurgling noises in his belly, which is exactly what we want to hear. Keep it moving Noah!! Maybe later this week they will be able to mix some of the Pedialyte with formula and see how well he will do with digesting the formula. The doctor yesterday said that when his bowels pick up and start moving a little better, then the bone marrow team will speak with the neurosurgery team to find out their criteria for internalizing the shunt. If Noah can keep up the great work that he has been doing this weekend, then maybe they will look at putting the shunt in sooner than we were expecting. We hope that this time it works. He has been waking up a little more often now since they decreased the dose of his sedation medication. It is nice to see him open his eyes again after over a week of having them kept closed. His sodium levels are normal again and he is not that puffy anymore. We are so grateful for the improvements that he has made over the last 4 days. He has such a long way to go, but if we have to take small steps to get there instead of giant leaps, then it doesn’t truly matter as long as we get there, right? We hope and pray that we get there…sooner rather than later. As for his engraftment labs to show the percentage of donor cells, I think they are going to wait until next week to do them, but we should find out for sure later today. Please continue to keep praying for Noah. Your prayers have made a difference for him this past weekend, and we owe such gratitude for all of the support and prayers. Thank you. We also want to make a special request for prayer. Yesterday late afternoon we lost another PBMTU kid. Ryan died after a very long & painfully difficult struggle for his life after receiving transplant for his cancer. Ryan was in the PICU both times that Noah was there and we got to know his mother well. We ask that you will remember this family in your prayers, that they will find some peace. We ask that you will continue to remember the other 3 PBMTU kids in the PICU right now, Juan, Travis, & Nolan. We also ask that you remember the other PBMTU kids and families too. It affects all of us on the unit when a child is lost, some more than others, but everyone gets a huge slap of reality knowing very well that at anytime it could be their own child. Thank you and God bless.

Friday, April 25, 2003 5:10 PM CDT

Entry 81---April 25, 2003 Post Transplant Day 65/ Post Transplant Boost Day 3

Well, anyone have any wood handy, because Noah has had a nice calm day without any major events. If so, please knock on it for us. Despite all of his present complications that we are trying to tend to, Noah has been pretty stable today. He has had some minor high blood pressure issues and is receiving some hypertension medications for it. However, he is tolerating the Pedialyte at a very low rate per hour. He is up to 3cc/hour (roughly ½ a teaspoon an hour) and has not had any major discomfort from the feed thus far. He has lost a lot of his excess fluid and is not near as swollen as he was. He has not been spitting up any blood today, which is what we want to see. We are so delighted to bring the good news for once, even if it is only about tiny baby step improvements. He was running a fever today, but is better now…not sure what that was from. Thank you so much for the wonderful prayers! THANK YOU, THANK YOU, THANK YOU!!! As we do each day, we again ask that you will continue to pray for Noah and his healing. We want to make special requests for prayer for some PBMTU kids that are in the PICU right now trying to make it back onto the bone marrow unit but are too sick yet. Please remember Nolan, Travis, Jose, & Ryan in prayer, along with the other PBMTU & Krabbe kids. There are so many that need so much prayer that we cannot remember them all. We also ask that you remember those families who have lost their children, if only you could try to put yourself in their positions, it is such a hard place to be…we cannot imagine losing Noah and the feelings of great loss and pain that we would be dealing with. We have experienced the threat of losing Noah, and that was enough to turn our lives upside down. So we want to remember them as well. We will list some web pages below that you may be interested in visiting. There are way too many to list, but these are just a few of the ones that we visit weekly to offer our support and to catch up on their progress:

www.caringbridge.org/hi/twilliams
www.caringbridge.org/co/nolanramsey
www.caringbridge.org/ia/jeremiah
www.caringbridge.org/ga/brentr
www.Krabbes.com

Thank you again for the prayer and support. God bless and have a great weekend.

Thursday, April 24, 2003 6:33 PM CDT

Entry 80---April 24, 2003 Post Transplant Day 64/ Post Transplant Boost Day 2

Nothing truly eventful happened today, which actually is probably more of a blessing, as exciting days are typically exciting because they are full of negative events rather than positive. So, Noah chose to behave himself today and kept his tendency to stress his parents out at bay. Thank you Noah from Mom and Dad! There was one thing that we were told during rounds this afternoon that was a little alarming. The doc told us that they want to draw some blood cultures to check for a possible “bug” that ‘may have’ been transmitted to him via the donor stem cell boost that he received on Tuesday. Again, we were told, “This is pretty rare, and occurs about 5-10 times a year out of all the transplants they do here on the PBMTU”!!! RARE, don’t say that word because Noah always seems to make a point to prove them that rare things CAN & DO happen! Maybe this will one day work to our advantage, but until then we seem to be going in the other direction and we don’t like to hear that word anymore. He was running a fever this morning, but it may be due to the graft vs. host (GVH) rather than a sign of infection. We will just have to continue to watch it. He has lost a little bit of water weight, but still remains quite puffy. He coughed up some blood, but it was not fresh blood, which means that we don’t see any present signs of active bleeding. His sodium has remained pretty stagnant today and has not dropped any further, so it still remains elevated. At least it is not going up again though. His Ativan sedation medication dose was reduced and he actually woke up crying a little early this morning. It was good to hear since we have not heard a peep out of him for several days, but we don’t like to hear him cry either. I guess we are becoming difficult to please. Noah will be started on Pedialyte tonight through his feeding tube. Hopefully he will tolerate this and he can be moved over to formula soon. Thanks for the support and prayers. Thank you to those of you who have sent us greeting cards. It is nice to get some supportive ‘fan’ mail once in a while. We ask for your continued prayers for Noah and the other PBMTU & Krabbe kids. God bless.

Wednesday, April 23, 2003 9:51 PM CDT

Entry 79---April 23, 2003 Post Transplant Day 63/ Post Transplant Boost Day 1

When are we going to be able to report some good news for a change. We are so weary of writing journal entries about Noah’s days that are so glum. However, until he turns a corner for the better, we have no choice other than to not write about what is happening altogether and we won’t do that. So I suppose you can guess where I am going with this one…Noah did not have a good day, again. Although his sodium level continues to come down, which is very good, it remains higher than normal. However, he has gained so much weight in excess fluid, over 2 pounds of fluid in just two days! He is one puffy baby! This can become serious if it is not released, but the diuretic that was given today did not work and he did not have hardly any wet diapers today. His blood labs concerning his kidney function look good, but he seems to be having some serious fluid absorption issues! It is already 10 P.M. and he is still so swollen! He is receiving another dose of the diuretic after receiving a large amount of plasma, so hopefully this will help. As for the spitting up blood issue, well he continued to do this most of the day, and the blood was no longer old looking. It became fresher looking as the morning went by and we became quite concerned. The nurse took a sample into the doctor’s rounds so they could see it, and finally they came around to see Noah in the afternoon to assess him. They wanted to see where the blood was coming from so they decided to slip another feeding tube through the other nostril and have it travel down into the stomach, and then pull out some stomach content to see if it appeared bloody. When they tried to he got really irritated (don’t blame him one bit!) and began to lose oxygen in his blood. He desaturated and turned a little dark in color. Fortunately the doctor was at his bedside. More oxygen was put on him and he slowly calmed down, turned pink again and his oxygen saturation began to return to normal. They have given him a lot of blood products today too, which has increased the amount of fluid he has taken in, but because the diuretic was not effective, he never got rid of any fluid, so he remains puffy. We will have to wait and see what tomorrow brings us. Until then we continue to pray that his donor cells will kick into high gear and he engrafts this time completely. We also pray that his other complications will soon resolve. Thank you for all of the prayers and support. A HUGE thanks goes out to the Kapi’olani PICU staff for your phenomenal support you have given throughout this entire journey. MAHALO GUYS & GALS!! We will never be able to truly show just how grateful we truly are. God bless you! Thank you to Uncle Chuck & Aunt Martha for the financial support too! God bless you too! We truly appreciate it. Thank you. We ask that prayers continue to go out for the other PBMTU kids, Krabbe kids, and families who have lost their kids to these horrible diseases. God bless.

Tuesday, April 22, 2003 5:44 PM CDT

Entry 78---April 22, 2003 Post Transplant Day 62

Okay Noah, do your thing buddy, GROW DONOR CELLS, GROW!!! Noah has been really sedated the last couple of days, not waking up even for diaper changes, when we turn him over or sit him up. He has been spitting up some blood today, and we are not sure of the source, but we feel it is most likely from his esophagus or stomach. He had another chest x-ray done and his lungs are looking a little cloudy, but he is retaining a lot of excess fluid. He slept through his transplant boost today. We hope that this rest will be of some benefit. His sodium has still been running quite high today, and he is still having his IV fluids managed & manipulated to try and help reduce the sodium level slowly. We thank you for the special prayers for Noah, and continue to pray that this time around Noah will do well and not have any more complications. We also pray that the complications that he presently has will resolve real soon. Most importantly we pray that Noah will have no pain and suffering. God bless you Noah!

Monday, April 21, 2003 8:49 PM CDT

Entry 77---April 21, 2003 Post Transplant Day 61

Noah keeps us on our toes. Last night he developed a blood sodium level of 170, and it was rechecked 3 other times to confirm that it truly was that high. The normal range is around 135-145. The docs were puzzled as to why this happened and had the Endocrinology team of doctors come by to help figure out why this was happening. Besides the bone marrow team, Noah has been seen by Neurosurgery, Pediatric General Surgery, Neurology, Cardiology, PICU Intensivists, Gastroenterology, and now Endocrinology! That is how many complications he has had,…so many that he has required being seen by all of these specialty areas in the last couple of months! Noah what kind of record are you trying to set? Endocrinology came in and looked at him, told us that this could be a quite serious issue that may place him in the PICU again, and that this high sodium level might be caused by Diabetes Insipidus. However, the fact that this came on so sudden is not typical of D.I., which is encouraging. Later in the afternoon they concluded that it was most likely due to a diuretic that Noah received yesterday, which caused him to void out a lot of fluid. So the plan for now is to stop the IV nutrition (TPN) and give him IV fluid with reduced sodium in it, check his blood sodium levels every hour to monitor the trends, and hopefully see it gradually dropping. It is too dangerous if it drops too fast, so they are trying to be cautious and have it drop slowly. Hopefully it will be back to a normal level within the next day or so and we won’t have any future problems with this again.

Noah received his third dose of ATG and tomorrow he receives his ‘transplant boost’ of the remaining donor stem cells. Then we will have to wait a few weeks to see what happens with this. He is still receiving the medication to help get his gut moving again, but they remain relatively still right now. However, because he is also getting Benadryl with this medication, he is able to rest and not have the discomfort that he had before, which is good. We are hoping that things start moving soon so that we can attempt the tube feedings again, and then later have his shunt internally placed.

Thank you for all of the prayers this weekend, and we ask that you will continue to keep him in your prayers, as well as all of the other PBMTU kids.

Sunday, April 20, 2003 6:33 AM CDT

Entry 76---April 20, 2003 Post Transplant Day 60

Happy first Easter Noah! This is an important day for our family to remember; because it is the day that Christ conquered death and rose from the grave to sit at the right hand of the Father. Without the resurrection there would be no life everlasting only sin and death. This event is the bedrock of Christianity and in our time or sorrow and fear a great comfort. This miracle can get lost at times in the myriad of consultations and medical predictions, the sleepless nights and pity parties at his crib side. Today we are going to rejoice in the miracle and be thankful, we are going to take a break from worry and sorrow and rest in Christ arms. We can’t go to sunrise services but we can open the blinds to Noah’s room, and as the sun rises and bathes his crib with light, we will be reminded of how He conquered darkness and brought light into the world. Tomorrow will be another day of worries and we will probably forget the silent hand on our shoulders as we hover and worry over Noah, but today we are going to try to remember His grace and be uplifted by it. We have placed Noah in His hands now, there is a do not resuscitate order on him and we have made the arrangements to donate his organs so he may help another family in need. We will pray the Lord lets us keep our precious Noah, but we realize that with every passing day that dream is becoming harder to keep in our grasp. For now we will wipe the sweat from our brows and strain against this illness for another day never forgetting that God weeps too.

Saturday, April 19, 2003 8:48 PM CDT

Entry 75---April 19, 2003 Post Transplant Day 59

There have been no real changes from yesterday’s events. The attending doc on call this weekend came in to share what he and the GI doc believe would be best option for Noah’s abdominal problem. They have decided to start him on another medicine to increase his gut motility without starting tube feedings. When listening to his belly, basically nothing can be heard, which means that his intestines are not moving at all. So they hope to get them moving more first and then start tube feedings afterwards, thinking that maybe this method will work for him. There is no word of placing a permanent G-tube, because of the requirement of going to surgery. Noah has been sleeping most of the day, which we feel is best so that he can rest comfortably. He got his first dose of ATG today, and will receive two more days worth before receiving the left over donor stem cells on Tuesday. We were told that they thought the dose of ATG Noah received a few weeks ago to help increase his donor cell growth would do the trick, but as we now know, it didn’t. The doc said today that basically we are in uncharted territories right now with Noah, and we will just have to take things a day at a time, and from here on out a lot of things are going to be experimental. Experimental…we wish that Noah did not have to be the lab rat and that there was more certainty behind these medical interventions. We appreciate all that the staff of docs and nurses have done for us here at Duke, despite the few mishaps, we are so blessed to have had the opportunity to bring our son here and have them do everything they know to try and save his life. God bless them for all that they do. Thank you for all of the prayers.

Friday, April 18, 2003 4:36 PM CDT

Entry 74---April 18, 2003 Post Transplant Day 58

Well, we have reached the eleventh hour. It came unexpectedly this morning when the doctor told us that Noah was losing his engraftment. We have been left with two options…One, take him home and let the illness take him or Two… give him three days of ATG which will wipe out his current blood supply, and then give him some left over donor cells to see if it will help. We chose the second option. He will be getting his second and last bit of donor cells on Tuesday, and we should know if it helped in about three weeks. If it doesn’t take, then we will be out of options, and will have to take our son home to die. This has been a crushing blow for us. Not one thing has gone well since we have been here. We use to pray so hard for Noah to get better and now we can only pray that His will is done. We hope this last shot of cells work, but to be honest there is not a lot of hope left in the old cellblock. We thank everyone for all the support and prayers, and will continue to pray for a miracle.

Thursday, April 17, 2003 8:07 PM CDT

Entry 73---April 17, 2003 Post Transplant Day 57

We have come to the conclusion that Noah just doesn’t do nights. It was a rough night again for mom. Noah was doing fairly well until midnight when he began to wake crying about every 15-20 minutes. He began having more frequent vomiting episodes throughout the night of old blood. We had been asking for something that would help reduce his nausea and vomiting, but since he was already getting the sedative every six hours, they thought that would be enough. However, it was not doing the trick. Finally today he was started on a medicine to help reduce any nausea that he has, which will hopefully decrease his vomiting. Every time he vomits he risks aspiration, which means that some of it gets sucked down into his lungs and can cause a severe pneumonia. He dropped his oxygen saturations after one vomiting episode and required a lot more oxygen to help keep him ‘pink’. His tube feedings remain off and he is only getting some medications through the tube right now. The doc asked a speech therapist to come in and assess his ability to suck, swallow and his readiness to have a swallow study. She said that just with her quick assessment of him today that her professional judgment is that it would be harmful to try a swallowing study and we should consider a G-Tube. The swallowing study is done by having a patient swallow at least an ounce of fluid and then do a chest x-ray. The x-ray will show if the fluid was effectively swallowed or if it was aspirated into the lungs. However, Noah is not even really sucking right now and would not even take an ounce without having it squirted into his mouth, and that is dangerous and not as effective. So she recommended that the Gastroenterology team come in to evaluate Noah and try to find the cause of all of his GI complications. They will likely be in to see in tomorrow morning and we hope that they will at least be able to give us some answers. We don’t want Noah to have a G-Tube placed, because it is a permanent feeding tube, which means he would be tube fed even when we go home. However, we don’t want him to suffer anymore and if a G-Tube will help him, then that is what we will do. It will of course be another surgery, but what do we do? They might decide to do an endoscopy, in which a scope is placed down his throat and into his stomach to see what is going on inside that would lead to his having old blood inside. We don’t know for sure, but that was mentioned to us today, so we could be prepared for that. He will be required to be paralyzed, sedated, intubated and placed on a ventilator again for that procedure. So whatever they decide to do, it will definitely be better than sitting here on our hands waiting and watching him be so miserable. That has been one of the most difficult things for us to deal with, because we are totally helpless. We pray that the newest medication will help prevent him from getting sick anymore and that the GI docs can help find the source of the problem with his belly and he will soon be out of any pain and discomfort. In the meantime, we pray that he will have a better night and get plenty of good rest, and us too!

Wednesday, April 16, 2003 6:44 PM CDT

Entry 72---April 16, 2003 Post Transplant Day 56

HAPPY 7-MONTH BIRTHDAY NOAH!!!! WE LOVE YOU SWEET BABY!!!! Yesterday, Dad kindly stayed with Noah while Auntie and mom went out to one of the shopping malls for the day. Noah’s tube feeding was started up again at a very slow rate again, but this time without the medication that causes an increase in gut motility in hopes of diminishing the discomfort that Noah was having last week during the feeding trial. He was all right for the first day of feeds and the doc decided to increase the rate slightly. Throughout the day he has been getting sick once again, vomiting old blood and we feared at one point today that he may have aspirated some into his lungs because he was dropping his oxygen saturations and unable to get them back up to the normal level without a lot of oxygen therapy. The doc stopped his tube feeding again and a chest x-ray was done. We have not received word on how the x-ray looks, but they have not been in to tell us any bad news, so we are assuming that hopefully he is fine. However, his tube feedings remain off for now and we are back again waiting to get his bowels functioning so that eventually he can have his shunt internalized, but as to when this will all take place remains a HUGE mystery. We continue to pray that God will help Noah get back on the path to recovery soon and allow his little body a break from all of this pain, discomfort, and stress that it has been under for so long. We continue to eagerly wait for the results for his chimerasm test showing the percentage of donor cells that Noah is growing. We should likely know in a couple of days. We pray that his donor cells are outgrowing his own bad cells this time. GROW THOSE GOOD DONOR CELLS NOAH!

Auntie Angela is on a plane right now, on her way back home to California. Thanks to Uncle Mark and cousins Logan and Jake for loaning her to us for a few days. We will certainly miss her and all of the great help that she provided us during her stay. THANKS AUNTIE!!

Thanks to everyone for all of the prayers and support over the last few months. We cannot say enough how much we appreciate all of the encouragement. Thanks for all of the prayers for all of the other kids and families on the PBMTU (and the three bone marrow kids in the PICU right now who are very sick), and for all of the Krabbe kids. We ask that you will continue to remember Travis, Brent and Nolan as mentioned in the last journal, and to include the other kids: Jeremiah, Elijah, Alex (girl), Ryan, Hermes, Aristophanes, Alex (boy), Harmen, Gregory, Kendall, Juan on the bone marrow unit. There are so many other Krabbe kids that we cannot name them all here, but please pray for them all, including Elly, Maddie, Makayla, and Victoria. Please check out the other Krabbe web pages by visiting the link listed below at www.Krabbes.com God bless and we pray that you all will have a wonderful and blessed Easter holiday.

Monday, April 14, 2003 7:18 PM CDT

Entry 71---April 14, 2003 Post Transplant Day 54

Where do I begin today? Well, Saturday night mom stayed with Noah since she was able to get a long nap in that afternoon. Noah was doing much better since the feeds were stopped and was able to rest more comfortably. Saturday night he slept ‘like a baby’ and mom was so well rested that she tossed and turned all night long. Finally when she got to sleep around 6 a.m. then Noah decided to wake up for a while, but again returned to sleep and mom rested for about 3 hours. Yesterday afternoon Auntie stayed with Noah and mom and dad got out for a while. Auntie decided to stay the night too and we were so very elated at the thought of actually being able to get a full nights sleep without any IV pumps or monitors beeping or suctioning. Unfortunately, Noah had other plans…once again, he tends to do things HIS way no matter what plans we may have in mind. Around 3 a.m. we received a call in the hotel from Auntie Angela saying that Noah’s blood pressure dropped to 60s/20s! His normal is 90s-110s/ 30s-50s, so this is very low for him. She said that the PICU staff was on their way over. STOP THE TRAIN…TIME OUT!! Mom asked Auntie to inform the large crowd at Noah’s bedside not to touch him until she arrived. At this time his blood pressure had increased back up to 70s/20s, a slight improvement but still on the low side. He was stable enough for them to wait and we did not want them to start poking and prodding, as we know they are quite eager to do at times. We made it over to his room very quickly and found out what exactly had happened. Noah received his regular dose of Ativan, sedative to help him relax from the Krabbe, but he also needed to receive Benadryl as a pre-medication for a powerful anti-fungal that he receives. If the Benadryl is not given, then he may develop a rash from the other medication, so this is always administered just in case. Well, ideally he should not get both Benadryl and Ativan at the same time, because they are both sedating and can cause him to be very sleepy. However, sometimes it just works out that they are given around the same time. Noah was apparently in a very deep sleep, had floppy tone and was not responding when trying to wake him up. This is when his blood pressure dropped. The fellow doc was paged and she came in to place him onto his back, a position in which he screams and does not tolerate, but he remained asleep. She sat him up and he did finally begin to cry a little. Auntie then called us to let us know what had happened. Noah was given some IV fluid and then later some Albumin too. His pressure slowly began to rise back to normal after a couple of hours. He was also placed on a Dopamine drip, and will remain on it for a while. It is a lower dose and will help his kidneys to process fluids better by increasing the blood flow to them. In larger doses this medication is used in more emergent situations to help maintain a blood pressure. After we were satisfied that he was stable and everything was okay, we went back to the hotel around 6 a.m. to try and sleep some. What a little booger he is!! It is no wonder that we have not developed any ulcers by now with all of the stress he has placed us under. We were able to finally get some sleep, the best sleep we have had in a long time. We came back over around 3 p.m. to relieve Auntie, and sent her over to the hotel to get some sleep. Noah has slept well since we have been back this afternoon and has not had any other problems. He has developed Thrush and is receiving a special purple dye type of medication that is placed all inside his mouth for three days and then it should help him get rid of his thrush. He looks like he has been eating purple ink! His tube feedings are still off and may be started up again tomorrow. Hopefully this third try at getting his belly going will be successful.

We owe a BIG, BIG THANKS to Auntie for helping us out so much during her stay. She shared with the nurse today that she is getting a taste of what we are dealing with. Noah says “Mahalo” to his best buddy Heidi for making him a special bunny, which she had specially named ‘Aloha’. It is so very cute! Thanks Heidi from mom & dad for all of the comfort foods and taking such good care of us. Thanks for the prayers and support. Please continue to remember us in your prayers, as well as all of the other PBMTU kids, both inpatient and outpatient. We have included some special requests below. God bless.

Please remember Travis, who continues to fight so hard for his life, but remains in the PICU still very sick. He just celebrated his 15th birthday last Friday. His web page is www.caringbridge.org/hi/twilliams

Please remember sweet baby Nolan and his family. He was diagnosed with Hurler, another metabolic disease and was transplanted last week. He had a big scare last week, which required him to go to PICU, but is now back on the PBMTU. www.caringbridge.org/co/nolanramsey

Brent was transplanted some time ago and is still growing those cells. He needs encouragement and support as well. He is such a sweet young man and always makes an effort to stop by at Noah’s window to wave and say ‘hi’ when he is out in the hall. www.caringbridge.org/ga/brentr

There are a lot of other kids who need just as much prayer as Noah, and some maybe even more. Please visit www.Krabbes.com and click on the various web pages for the many Krabbe kids to learn more about them, their families and the incredible journeys that they have made. Each has an inspiring story that will touch and even break your heart, and hopefully encouraging everyone to live with a greater appreciation for life, health, and family. God bless each child and their family.

Sunday, April 13, 2003 1:03 AM CDT

Entry 70---April 12, 2003 Post Transplant Day 52

Another weekend has arrived, and they are usually much less hectic than the weekdays. Noah’s feeds were stopped yesterday around noon, but remained a little fussy off and on throughout the day and into this early morning. However, it was nothing like he was this week. For whatever reason, he was never given his Mylicon drops last night and so he did not receive his first dose until late this morning. Although his tube feedings have been stopped temporarily, he still has his feeding tube in place for medications, but the last few times that he has received meds through it, he has begun having dry heaves afterwards and trying to vomit. Today the doc told the nurse to try pushing the medications through the tube very slowly. It takes up more time for them, but it has seemed to help do the trick. He occasionally will still have abdominal cramping that causes him some discomfort, but again, it is nothing like the pain and discomfort he was having during the week. The big problem that we face is that he must be able to get his gut up and moving again in order to decrease the abdominal pressure, which will allow for his internal shunt to be placed, finally. However, he has had SO much discomfort and pain with the tube feedings thus far, that we are not sure how the docs are going to be able to help solve this problem. We have a lot of faith in them and trust that they will arrive at a solution eventually, but in the meantime we have to wait (patiently????), and the longer Noah has his external drain (EVD) in place, the higher risk of infection. He still remains quite puffy and swollen, and his weight was up a bit today again. He had received some blood products this morning, which is part of the reason for his increase in weight, but we do worry about him having too much fluid.

Auntie Angela made it safely here very early this morning and we were delighted to see her again!! As always she looked absolutely beautiful and we are so very glad that she was able to finally meet her nephew! She has been such a great source of support for us throughout this entire ordeal and has also signed up to be an official Hunter’s Hope Volunteer. THANK YOU AUNTIE for working so hard to educate people on Krabbe’s and other leukodystrophies, so that we can help other families and kids. She stayed with Noah a lot today so mom and dad could get some much needed sleep and a nice meal. What a blessing! Thanks to the many supportive guest book entries this week. We have been very stressed and have confronted a lot of difficult emotions these last few days and have needed to read the encouraging entries to help keep us focused. We cannot say enough how much we appreciate the prayers for Noah. At times we are not certain that they are doing much for him, but he is still here, alive and staying strong despite the constant hurdles that he has had and is currently having to face…so they must be at least doing something. Is it enough? Of course not, and to say that it is would be a lie…we would love for him to be completely healed from his pain & discomfort, healthy, ‘normal’, and on his way home without any complications; but that is not what has happened and we don’t know why. At this point we simply only want him not to suffer anymore. However, we still have him with us and we have met families here who are having just as difficult a time, and some having much worse a time than we are. We acknowledge that we are not an isolated incident and that there are many kids and families suffering. We ask that you will continue to remember Noah in prayer, and not to forget the other PBMTU, Krabbe, & all other kids and families struggling with life threatening diseases. We will attempt to do our part by staying strong, focused and having faith that we will ‘all three’ make it out of this soon. We know that we are blessed to have each other to lean on and to have so much support behind us. Thank you so much for listening to us vent, complain, whine, and cry these last three months. We will continue to do so and we thank you for continued prayer and support.

Saturday, April 12, 2003 0:11 AM CDT

Entry 69---April 11, 2003 Post Transplant Day 51

We were up again nearly all night with Noah. Around midnight he received another of his scheduled doses of Ativan, sedative, and he slept off and on for about two hours. Then a special x-ray was done of his abdomen to see if there was anything significant that would show up as a cause for all of his irritability and discomfort for the last three days. It was slightly irregular, but more significant was a small pocket of air stuck in a certain part of his bowels that is known to cause some discomfort. We are not certain if this is the cause for his constant crying lately, but it certainly could be. His tube feedings were turned off at midnight and turned back on about 3 a.m. He woke up with the process of getting the x-ray and then did not stop crying and moaning again until he was given more Ativan at his next scheduled time around 6 a.m. We both stayed over since he was not doing well. We spent most of the day crying and expressing our feelings of doubt, uncertainty and guilt for putting him into this situation; so we decided that it would be easier to survive the night together. Noah finally went to sleep somewhere around 6 a.m. and we decided to try and get a little sleep. Noah was back up and crying again around 8 a.m. and mom sent dad back to the hotel to get some ‘good’ sleep so that at least one of us would be worth something later in the day. His feeds were once again shut off and it was decided to let him have a rest for the weekend. He was started on Mylicon drops for gas, not sure that it would really help, but we asked for them to at least give them a try. He seemed to have a little better day, but still required quite a bit of suctioning and oxygen to keep his saturation level up above 93%. He was also started on another medication to help decrease his mucous production, which will hopefully help cut down on the need for so much suctioning. The doc muttered again that he needs to have a swallowing study done soon, and that he may eventually require a ‘G-tube’, which is a feeding tube that is placed directly into the stomach and allows for feeds to be given in bolus form. We don’t want Noah to be on tube feedings forever, but if he does not recover his ability to suck/swallow, then it looks like we won’t have too many options. This is still down the road, and it would require another surgery, but it is just another thing that we have to ‘keep in the back of our minds’. Thanks so much for all of the encouragement, and for praying so hard for Noah, as we are not able to find the strength to do so as much as we used to. God bless.

Thursday, April 10, 2003 10:36 PM CDT

Entry 68---April 10, 2003 Post Transplant Day 50

These last couple days have been extremely emotionally trying for us. Noah was up again all of last night crying and moaning, requiring frequent suctioning and oxygen. He is absolutely in total misery. What have we done to our poor baby boy? Why have we put him through this? If he does not make it, will it have truly been worth it for him to have suffered so much? He looks and acts nothing like the sweet baby boy we carried into this room just two months ago today. If it be God’s will that he dies and does not make it through, then I pray that he take him now and prevent him from suffering anymore. We are at a loss as to what to say or think anymore. We feel that we have been fighting so hard with the docs for what is ‘best’ for Noah, but it seems that something else bad ends up happening. We were told today that the upper lobes of Noah’s lungs are ‘cloudy’. He is on antibiotics already to cover any possible infection, but it still is not good news. The doc suggested that Noah may not be able to effectively swallow anymore and will need a swallowing study to determine this for sure. It does not matter right now as far as his nutrition is concerned because he is being tube fed, but he may be aspirating his mucous. They are not certain and neither are we. There never seems to be any ‘definite’ or ‘certainty’ in anything that we have faced, except that he is physically miserable and we are suffering emotionally by all of this. He was started on Albuterol breathing treatments as needed to help open his airway a little better. A neurosurgery resident stopped in and said that there is no definite plan of care or time frame yet as to when Noah will be able to have his shunt internalized. We want to make sure it is the last surgery that he has for a very long time and don’t want to rush them if they don’t believe that he is ready yet. Prayers are definitely needed and we are grateful for the prayers that have been offered on his behalf. We ask that you will continue to remember us in prayer, and the other Krabbe & PBMTU kids too. We pray Auntie Angela arrives safely tomorrow night! We look forward to seeing you. God bless.

Wednesday, April 9, 2003 2:17 PM CDT

Entry 67---April 9, 2003 Post Transplant Day 49

We have all had an extremely rough few days and Noah is not feeling very well at all. He is very puffy, swollen, either asleep or crying out in pain when awake, has so much mucous he sounds as if he is drowning in it at times and we are at his bedside every 15 minutes suctioning just to keep his airway open, and he does not seem to be getting better…yet. We are so exhausted and have become easily frustrated and irritated with this horrendous adventure. Noah has changed so much the last few months, and at times is unrecognizable from all of medications, treatments and therapies that he has been or is currently on. At rare times when he is quietly awake and we try to gaze into his eyes, it appears as if he is looking through us as if we are not there. We are halfway to our ‘going home’ time at 100 days post transplant, and yet we are so far from getting to the outpatient phase. Maybe it is a blessing in disguise, as we have only heard how grueling the outpatient phase is having to go to clinic every day and sit there waiting for hours; but we have tried to look on the ‘positive’ side of things for so long and after having to always face what seems to be the worst, over and over and over again, it becomes much harder to do. We will come out of this somewhere on the other side, not sure where and if we will all be there or not, but we will make it through. It is the getting there that is the hardest to endure.

Noah has tolerated his tube feedings thus far, and his rate was increased ever so slightly to 4cc/hour, just under a teaspoon/hour. He is having some difficulty tolerating his belly working again, but hopefully with time this will become less of a discomfort to him. He has required some blow-by oxygen because of the large amount of mucous in his airway. Occasionally the large amount of mucous will cause him to have issues with nausea & vomiting. We continue to remain scared of him actually aspirating when he vomits, because this can cause him to become very, very ill. He is not able to turn himself, and although he sleeps on his side, he tends to always have his head arched back and remains at risk for aspiration pneumonia. We have been able to ward this off by being at his bedside constantly to suction as needed. He has his EVD still that is draining off fluid and will have this in until they decide he is all right to have the permanent shunt placed again. He has had 4 surgeries in the last 4 weeks! He has been through so much and still he fights. What strength and perseverance he has! We are strong because he is strong. He remains on his IV nutrition until he is able to tolerate being on the tube feedings only. His sedation medication was reduced during the day, but left at the higher dose at night to help him sleep better. His CMV has negative for the ‘active’ virus. He will always have it, but it is dormant right now, which is good and although he remains on the Gangcyclovir for a short time more to be certain it stays dormant, he is now off of isolation and we no longer have to wear those annoying gowns, gloves and masks when we leave his room. Noah will have his next chimerasm blood test to check for the percent of donor cells next Monday morning. It will take nearly the entire week to receive the results from Dr. Wenger in Philadelphia, since his lab only does the testing on Thursdays. We pray that he is not continuing to lose his engraftment, but we won’t know for another week. We no longer look each morning to see if he has WBCs, because we know that he will, but we continue to look at whether his liver and kidneys are doing okay. Some of the kids have required dialysis for their kidneys because of the hit they have taken from the transplant process. Noah’s kidneys and liver are being a little overworked, but are hanging in there. We are fortunate not to have to worry too much about this as some of the other families have. We pray that we can begin to clear up some of these extra problems that have arisen over the last couple of weeks so that we can narrow our focus to just regaining his engraftment and getting his internal shunt replaced soon. Hopefully, he can then begin to recover from the entire trauma that he has been through these past few months.

We owe a big THANK YOU to Greg’s unit back home for renewing his assignment, allowing him to stay here with Noah and I. We want to say another huge THANKS for all of the prayers, encouraging words left his Noah’s guest book and financial help. We continue to ask for your help as we remain in the midst of this storm. God bless you. To Noah’s Auntie Angela coming from California on Friday, we pray for a safe and speedy trip and we look forward to seeing you after 5 long years!

Monday, April 7, 2003 8:38 PM CDT

Entry 66---April 7, 2003 Post Transplant Day 47

Noah had a relatively good night except for a minor scare when he dropped his blood pressure down to 70s/20s, which is well below his normal. He received a fluid bolus for this and began his morning looking again like the ‘Michelin man’, all puffy in his face, arms, hands and feet. It is such a delicate balance trying to keep his fluid regulated, and sometimes when he has too much in his system, he becomes so puffy and swollen and then requires a diuretic to get rid of some of the fluid. It is a continuous cycle that Noah goes through, because he gets blood products just about every day. He finally settled down and has had a quiet day today. The doc came around and said that his kidney and liver labs look better than the past couple of weeks, but are still a little elevated. This is expected due to all of the medications he is on that are metabolized by either the kidneys or liver. He was once again started on his trickle feeds slowly at 1 cc/hour and then increased this afternoon to 2 cc/hour, and Noah has tolerated them well so far. He is on a very low dose of the antibiotic Erythromycin, which has been shown to have a side effect of increasing bowel motility (diarrhea when normal doses of the medication are given) and this helps him digest the tube feeds better. So far he is tolerating the feeds and has not vomited yet related to the tube feeding. He has been sleeping almost around the clock since he got out of surgery on Friday morning because the PICU docs doubled the dose of his sedation med. We asked the bone marrow doc today to change it back to the lower dose, but he wants to wait a couple more days first, and suggested that the rest would be good for Noah anyhow. We don’t mind him sleeping, but we are eager to see him alert and awake more often. He has to be turned from side to side every few hours and he does not like this at all. He screams with discomfort and pain when he has to be moved, and so we feel okay about letting him rest and not so eager to hold him to keep from hurting him. He had a WBC count today of over 20, which is way above the normal high range, but he is still getting the medication that stimulates WBC growth, so they decided today to cut his dose in half. Again, we will have to wait a week before he has his lab drawn to recheck his percentage of donor cells. We are praying that the number will be higher this next time. GROW THOSE DONOR CELLS NOAH!!!! Dr. K (we call her “Special K”) promised two things, first, that Noah would not have any permanent damage from the increased pressure in his head and second, that Noah would not lose his engraftment. So we are listening to what she is telling us and counting on it to get us through.

We owe a HUGE thanks to the PBMTU staff for training on the use of Noah’s EVD drain so that he could get out of the PICU and return back to his room on the bone marrow unit. THANKS GUYS!!! We also want to say another BIG thanks to Noah’s Gammers who just left tonight to return home after her two week stay with us, and for helping us out by sitting with him in the PICU on the ‘night shift’. We are so very, very glad that you were able to see him again mom, even though you were not able to hold him because he was too sick! Thanks to her co-workers at Wal-Mart for making it possible for her to come and to those of you who have given us some extra financial support! We truly appreciate it more than you will ever know. THANK YOU!!!! We pray that she returns home safely! Thanks to Kelly for playing ‘taxi’ for us, and getting mom to and from the airport! We owe so many of you a big ‘THANK YOU’ for all of the prayers, encouragement and support that you have provided us in so many different ways, and we will never be able to mention you all here, but please know how much we appreciate you and that we are so very grateful for the kindness that you have shown to us. God bless you. Please continue to remember us in your prayers, as well as all of the other Krabbe kids, PBMTU kids and those who have recently lost their children; and we will remember to pray for you!

Sunday, April 6, 2003 5:24 PM CDT

Entry 65---April 6, 2003 Post Transplant Day 46

Noah has done well since coming back to the unit. They decreased the setting on his EVD from 5 to 3 and now he has been able to maintain a stable heart rate and keep his O2 stats up. For a while yesterday we were a little scared because he continued to vomit, but after dropping the shunt pressure settings he seemed to settle down. Now he has been sleeping away the day. It is obvious that his external drain is causing him some pain by his reaction every time we move him. Hopefully that will settle down in the next few days and we will be able to hold him again. For now he looks remarkable stable which is nice to see after almost a month of problems, with three operations, and almost a three-week stay in the PICU. We appreciate all the support from family and friends. It’s nice to be reminded that you are not alone because when things get tough around here, it can feel like you are stranded on an island.

Friday, April 4, 2003 8:22 PM CST

Entry 64---April 4, 2003 Post Transplant Day 44

Well we left Grandma with Noah last night to keep him comfort, and he had a quiet night. His trickle tube feeding was stopped for surgery and he received his dose of ATG. We made sure to leave all of our contact numbers with the nurse yesterday and told her that we wanted to be able to go down to the OR pre-op waiting area with Noah. We would wait in his bone marrow room to be called since we are not allowed in the PICU during 0700 to 0930 in the morning for rounds. We were told that he was scheduled for surgery around 930. At 0830 this morning the Anesthesiology attending came over to Noah’s bone marrow room to get the consent signed and said that they had another case before Noah’s and that they probably would not call for him to come down for about another half hour. We were up and ready to go and Greg called over to the PICU around 0850 to see when Noah would be going down to OR and was told that he was already taken down. When we got down to the surgical waiting area we were informed that he was already in surgery! Then after the actual surgery we were called into a consultation room to speak to the neurosurgeon who informed us that all went well, but that the external drain is not coming out of his head, rather it is running down underneath the skin and actually exits through his chest…which is not what we were expecting. However, despite the shock that we were not informed prior to the operation of the surgeon’s plan, we understood after hearing his explanation that this was a better option than the type that Noah had previously. This type of external drain placement reduces the risk of infection and allows for it to be left in place longer than just a week, which should last until he is able to have his shunt internalized. We then returned out to the waiting area expecting to be called back shortly to the recovery area where we would be able to see Noah and travel with him back up to the PICU, and we waited there for about a half hour. Greg asked the waiting area receptionist about Noah and she informed us that he had already been taken back up to the PICU some time ago! We were quite upset and distraught! When we asked the PICU staff, they pointed fingers to Anesthesiology and added that they had made ‘special’ arrangements for us to come in during the prohibited hours in the morning…but they did not manage to tell us about this ‘special arrangement’. We let Dr. Kurtzberg know that we were unhappy about what had happened, she apologized and said that she would take care of it. We learned that the PBMTU staff is being trained today and over the next few days on how to monitor Noah’s external drain and this will allow him to be moved back to his room on PBMTU tomorrow!!! YEAH DR. K!!!! Saying that we will be very, very happy and relieved to be back in his room on PBMTU is a HUGE understatement. Noah is stable right now, but has been knocked out from the anesthesia all day. He appears really puffy and probably needs a diuretic to get rid of some extra water, but he is resting comfortably. He will start his trickle feeds again tomorrow. There really was not much else outside of his surgery that occurred today. We are pleased that it went all right and even more so that he will be back on the bone marrow unit tomorrow! Thanks for all of the support. Thanks to Greg’s unit back home for offering your special support and allowing him to stay here for Noah’s sake and mine. We will not hear anymore about his engraftment issue for another couple of weeks, but we ask that until then that the prayers continue to keep coming. As well, we ask that you will pray for the other PBMTU kids, Krabbe kids and the families who have recently lost their children. God bless.

Friday, April 4, 2003 1:51 AM CST

Entry 63---April 3, 2003 Post Transplant Day 43

Okay, every one in unison, “WHAT NEXT?” Noah seems to always answer that one for us, and again he has come up with something new to have his parents worry about. He had labs drawn at the beginning of the week and sent off to the same special laboratory in Philadelphia that ran the blood test, which confirmed his Krabbe diagnosis. This recent sample was sent to check for the presence of the enzyme that he was missing, and it came back showing that he already had some enzyme present, on the lower range of normal. This is a good thing because we can be sure that it should be starting to break down those toxic chemicals in his brain and prevent further damage. HOWEVER…his other lab work that was testing to see the percentage of donor to his own cells was lower than expected last week, at 46% (expected to be above 90%). It was repeated this week and showed that his new donor cells have dropped down to 32%! This is a BAD THING!!!!!! This means that his new donor cells are being overrun by his own ‘bad’ cells and his ‘good engraftment’ is fading away as his own cells are taking over again. So although he currently has enzyme, he must hold onto his new donor cells to make the enzyme. As he loses the donor cells, he will also lose the ability to make any more enzyme, and what he has now would eventually be lost as well. Then he would be once again “back at square one”. (This is another phrase that we have had to use way too often and we are getting tired of it). We were absolutely devastated to hear this today, as we were hoping that at least this would go right for him despite all of the other issues he is having. He will begin to receive doses of ATG again, as he did with chemotherapy to kill off some of his white blood cells and hopefully allow his donor cells a better chance of trying to outgrow his bad cells. This immunosuppressant therapy will begin tonight and will probably be repeated as needed weekly. He will also begin on another type of special medication to help kill off the “T cell” WBCs that are attacking his body, and hopefully will also help him work through his graft vs. host disease. Both of these drugs in combination will hopefully allow his donor cells to eventually outgrow his old bad cells. We will have to wait about two weeks more before we will be able to find out if this is working or not.

His EEG again showed that he had the ‘potential’ to have seizures, that his threshold for having seizures was much lower than the average person, but he is not actually having any yet. A lot of the medications that he is on can cause seizures. He will not be placed on any seizure meds at this time, which is a relief. He also had another head CT scan this morning to check his brain/fluid status. There was some air bubbles noted in the last CT scan that had gotten inside where the fluid is supposed to be via the shunt, but these air pockets are shrinking and today’s looked better. He is still vomiting despite the very slow trickle feeds and the new special formula. He is going to be started on another medication to help get his gut moving, and maybe this will help him digest the formula better and decrease his nausea and vomiting. As for his CMV+ status, his nasal passages and urine have been clear of it. The docs think that it is only the CMV DNA that is being detected in the blood lab work and that the virus is not actually active right now, which is another relief. However, he continues to be on contact isolation and we must gown and glove to go into his room. The even better thing about this is that if he ever is able to have his shunt internalized again, then at least this won’t hold him back from surgery. The neurosurgeon said that they do not operate on anyone who is CMV+. His lab work reflecting his liver and kidney function are better today than yesterday, despite the elevated values. His WBC count today was 11.8, but again a lot of these cells are his own bad cells.

As for Noah’s neuro status, well he will be sent to the operating room in the morning to have the externalized drain removed and another replaced, since they are only good for about a week. They are changed so frequently due to the high risk of infection if left in too long. Hopefully this will be the last before returning to the OR to have a final permanent shunt placed in the near future. It will all depend upon if his abdomen begins working again the pressure relieved.

There are so many things going on all at once right now. We are becoming so numb from all of the emotional ups and downs that it is hard to know whether to be scared, hopeful, angry, to cry, have a pity party for two, laugh, worry, or just scream. Sometimes it is all of the above wrapped into one huge emotion explosion that eventually occurs after our maximum capacity for all of this stress has been reached. We have reached this a few times already. We think we have had our fair share lately and we don’t really want any more at this time. When will it end?

Thursday, April 3, 2003 2:56 AM CST

Entry 62---April 2, 2003 Post Transplant Day 42

Another hectic and emotionally crazy day for us has almost come to a close as night sets in again. Last night Noah began vomiting, three times total throughout the night and early morning, and his tube feeding was turned off. So he was back to the proverbial “square one” again. As well, Neurology stopped back in to once again bring up the topic of Noah’s potential to have seizures and the ‘need’ to look into this further. We continue to argue that he is not having seizures and will refuse to consent to giving him any Phenobarbitol, a seizure med that will absolutely snow him and mask his neuro status. We gave in to having another EEG done, but this one running over an entire 24 hours and not just 20 minutes. Maybe this will prove our point once and for all, and we can be done with this whole ‘seizure’ mess altogether. However, IF he is indeed having some small silent seizures and we are mistaken, then we can work on treating them with an alternative to the Phenobarbitol. The technician arrived this morning to place the multitude of electrodes on his head and began the EEG around 1000, and it will run until this time tomorrow morning. He had a special x-ray of his belly to check placement of his feeding tube since he was having vomiting episodes, and it is indeed correctly in place. The docs decided that maybe the formula that he was started on was ‘too much’ for his system because it contained complex proteins. They changed him to a new special kind of formula this afternoon that has the proteins in it already broken down, allowing his intestines to not have to work so hard to get back up and running. Since he has been on the IV nutrition for almost 2 months, his belly has not had to work at breaking down food. The intestine is like any other muscle, if it is not used for a while then it will atrophy. He has vomited once since being on this new formula, but only a very small amount. Hopefully he will tolerate it and his gut will start moving so that we can work towards getting the internal VP shunt replaced soon. His liver is still enlarged, but they are just continuing to keep an eye on his blood lab work values that are associated with liver function. Although these values are elevated, they are still not real concerned at this time. His lab work that monitors his kidney function was very high yesterday and is still somewhat elevated, but much better than it was. He has been sleeping literally almost all day and evening long, because some doc (not his bone marrow docs) decided to double his Ativan, his sedation medication to help him relax. However, we were surprised and quite concerned that his Ativan dose was increased, because it is so nice to see him awake. However, we are just guessing that since he had been a little fussy the last couple of nights they thought that he could use some extra help calming down. We are not as concerned about it for today since he must lay still for the EEG, but tomorrow we plan to question them as to why they felt the need to sedate him so much that he sleeps all the time. One of the Fellow doctors on tonight stated that Neurology thought that Noah may be building up a tolerance to his original dose of Ativan, and that this may be the reason for the increase. He also stated that Noah’s medicine regimen can always be altered to find the best ‘mix’ for him, but it will just take some time. He does occasionally wake with diaper changes. He awoke tonight and we found him to have what is called a “disconjugate gaze”, meaning his eyes were going in different directions! Yikes! The doc has not yet been in to see him, but we will certainly have to find out what he thinks on this one. Maybe one of his drug levels is too high in his blood and is causing a reaction. We were hoping to be back on PBMTU by now, but Noah has too much going on right now and unfortunately it will probably be another week. His EVD drain has been in almost a week now and will have to be changed soon, but because of the possible active CMV, neurosurgery is hesitant to do anything invasive at this time. We will only have to try and be patient (yeah right!) and pray that all goes well.

We met our new next-door neighbor on the PBMTU today who has late onset Krabbe’s disease and has begun his chemotherapy. Although it is nice to have someone who understands a little bit more about what we are dealing with so nearby, we wish that no other child would have to endure this terrible disease. Hopefully we can be of some comfort to them as they begin the transplant journey. We know that Noah’s journey has been rougher than that of some of the other transplant kids on the unit, and we hope that no one mistakes our candid way of expressing our transplant experience as being unhappy with our decision. We feel blessed that Noah could be transplanted and that it is a method that has been proven to spare the lives of Krabbe kids, who otherwise would not be here today. However, we want to share the entire experience and not just the good in it, because it is so very emotionally grueling to parents and an extremely physically miserable journey for the kids. Some fair better than others, but that is the way life is, but that is exactly what ‘it’ is, a chance at life…and that is all that matters to us in the end. God bless ALL of the doctors and nurses and other staff that have put Noah’s best interest at heart and are trying to save his life, despite our differences of opinions at times, we appreciate them all. Thank you for your prayers and your guest book entries that are so uplifting to us, especially during these very stressful past few weeks. Please pray EXTRA hard for us as we have just learned today that Greg’s unit back home is considering calling him back to work there, which will leave me here alone with Noah to try and endure this by myself. I cannot imagine this happening and we pray that those who have the ‘power’ to make this decision will put themselves into our situation and decide that it is best for BOTH of us to be here with our very sick son during this time. They should not fret because we plan to come back home as soon as we are able to with our son alive and well.

Tuesday, April 1, 2003 7:29 PM CST

Entry 61---April 1, 2003 Post Transplant Day 41

Wouldn’t it be nice if this was all a joke and we were just being fooled for today is “April Fools Day”. However, it is very real and it certainly is not funny. Noah began last night by again causing us to worry when his pupils were unequal, one bigger than the other. The PICU doctor came in and began messing with him, made him agitated and he began to wake up more. After that he whined and cried most of the rest of the night and required pain meds to try and make him more comfortable. The upside to all of this was that he was at least opening his eyes more and moving his arms a little more as well. This morning he had another head CT scan to assess the cause for the low heart rate episodes that he continues to have periodically, and this time mom and dad went along for the ride and made sure he was masked! Overall, the bone marrow doc is pleased with Noah’s progress. They increased his tube feeding rate gradually, and he is presently at 6cc/hour, which means that he still is only getting just over 4 ounces in a 24 hour period, but he has tolerated it so far and he will get more each day if he continues to do this well. As for his CMV+ status, we are still having to gown, glove and mask, which gets very old…fast! We are waiting for his urine lab results to come back, probably on Thursday, and if they are negative then we probably don’t have to do this anymore. As well, the neurosurgeon called early this morning to let us know that they are pleased to hear that his feeds are started and he is tolerating them well, but that because of the CMV status they do not want to operate on him in case it infects the internal shunt that they would be putting back in. However, he said that he would speak to the bone marrow team to see what they think about it before anything else is decided. When talking with the bone marrow doc about it, he mentioned that the CMV issue is not a big deal and that as long as it is not ‘active’, which they believe it is not active in his system at this time, then there is no reason why they cannot go ahead with the surgery once they feel that his abdominal pressure issue is solved. Noah’s chest x-ray that was done this morning showed that his lungs looked remarkable and no need to worry about pneumonia at this time, which is a big relief. He has not had many episodes of oxygen desaturation today either and has not really required the oxygen blow by, except when he cries and holds his breath briefly (vagal response). His WBC count is 6.6 today; keep grow those donor cells Noah!! His BUN is checked daily in his blood and reflects his kidney function. It has been on the higher side lately, but today it was very high. They are stopping some of his medications that are harder on his kidneys and are continuing to monitor this closely. This is just another number value that we are now watching, but we are pleased that overall Noah is holding his own and not giving in to the enormous stress that has been placed on his little body. Thanks for all of the prayers.

Monday, March 31, 2003 10:15 PM CST

Entry 60---March 31, 2003 Post Transplant Day 40

Okay, I admit it; recently I have spent a lot of time screaming at God. I have walked to the top of the mountain, and in the pouring rain, stood with out reached hands and yelled at the top of my lungs things like…. Why? How could this be possible and, how could this happen to such a beautiful child? I have wondered if I am being punished for my sins, knowing very well it doesn’t work that way, but the question still lingers on. I have had my quiet moments, digging through the dark, cluttered, and ugly closet of past sins. I have spent countless hours having one to one talks with Jesus, asking for forgiveness and peace. Sometimes I feel like Job, but I’m scared that I may not have his strength to endure anymore…

Last night we asked the Resident to schedule Noah’s feeding tube to be placed down in radiology as soon as possible in the morning so that the feeds could be started thereafter, since we wasted 3 days waiting for them to do this and the fact that it was actually ordered to be placed on Friday. The Resident was able to make arrangements for Noah to go down to radiology around 10:00 or 10:30 in the morning, so we were planning on making sure that at least one of us was over there to go down with Noah and the nurse, and especially to make sure that Noah’s mask was placed on him throughout the duration of his time outside of isolation. At 0930 this morning, when visiting hours are supposed start and rounds to be over, dad was at the door buzzing to go in and was told he would have to wait because rounds were still in progress. He waited 20 minutes and buzzed again only to be told that Noah was no longer in his room and had already been taken down to radiology. His first concern was that Noah’s mask was placed on and that if he was awake and crying, that Noah would not have one of us there to comfort him. He confronted the resident about Noah wearing a mask and she was not certain, but just after speaking to her, Noah was being wheeled into the PICU by his nurse WITHOUT HIS MASK ON!!!!!!! This was the same nurse that last time Noah was in the PICU in an ‘isolation’ room who left the doors wide open for several hours because the room temperature was ‘too hot’ and ‘she was burning up’! Does she not get it? Obviously not, and meanwhile she has twice now put our sweet baby’s life in harms way because of her negligence and carelessness! Dad was FURIOUS, but was very restrained, showing his deep concern without making a scene. Once mom was informed she ran over to the PICU to inform the nurse that she needed to leave Noah’s room and NEVER return!!! She is a dangerous nurse and her carelessness could cause Noah to have been exposed to something/someone infectious and even kill him, so this is VERY serious!! The most appalling thing was that she did not even seemed sorry about it or apologize for it. So we again reported her to Patient Relations and had another meeting, this time with the nurse manager for the unit, but it did not seem to us coming out of it to do any good. We again bothered Dr. Kurtzberg and we heard through the grapevine that she was definitely not pleased, however she was busy today with a conference so we did not actually get to speak with her.

As far as Noah’s health status, he continues to remain on some blowby oxygen because his oxygen saturations have been dropping into the 80s%. He has had a few heart rate drops, usually when he holds his breath. He has been really lethargic today and has less tone in his arms/legs. He was started on trickle feeds finally and seems to be tolerating them well so far, but again they are going so slow that we won’t be able to really see an intolerance until they pick up. His WBC was 5.2 this evening, which is good, but again we know that he is still growing some of his own bad cells still and is really only growing 46% donor cells at this time. He had another set of lab work taken and sent off to check this % and the results won’t be back until this next weekend. We hope to see an increase in the % of donor cells. However, the main concern besides his ICP and EVD issue and his engraftment, is that he again has tested positive for CMV, cytomegalovirus, and is on ‘contact isolation’. We must be gowned, masked, and gloved to be near him and still cannot hold or cuddle him because of the EVD. If this CMV gets out of hand it can cause a severe pneumonia to develop, which may kill him. He will be started on the Gangcyclovir again, which he was on the first few weeks after we arrived here at Duke. However, we have so many other things to worry about and as we mentioned before, we stopped asking ‘what next’ because something always seems to arise. Will it ever end? When he is finally dead and nothing else can touch him? This is getting to be so very old. We are to speak with neurosurgery tonight to find out what exactly their plan is for him, but it looks like he will be in the PICU for at least another week, and may need to go back to surgery to have another temporary EVD placed later this week until they decide to put the permanent internal shunt in. Please continue to send up prayers for him, because things seem to only be getting worse for him. He is miserable right now and we are helpless.

Sunday, March 30, 2003 4:56 PM CST

Entry 59---March 30, 2003 Post Transplant Day 39

This entry will be brief because not much has come about since the last. Noah remains in the PICU with the EVD drain coming out of his head, draining the excess spinal fluid and having his ICP monitored. They still are not sure what caused his abdominal pressure to be so high, but Dr. Kurtzberg contacted the bone marrow attending doc covering this weekend and told him to inform the neurosurgery team that they have two days to figure it out and then they need to begin action. She does not want Noah to have an external drain in due to the high risk of infection, and we agree with her to some extent. However, we also want to make sure that this problem is thoroughly researched and solved prior to taking him into surgery again and risk the same problem arising again. We don’t know exactly what to think about all of this, but are standing on guard at his bedside to make sure we are kept informed of everything before it happens. The feeding tube that was placed for his ‘trickling’ feeds was found to be “out of place” today and his trickle feeds stopped and the tube pulled out. So tomorrow they will take him down to put it in under what is called fluoroscopy, so that they can view it being put in and make sure it is in the correct spot, so that when he returns to the PICU he can be started on the trickle feeds again immediately. We wish they would have just done this in the first place, but again it seems that everything must be done the hardest way possible when it comes to Noah. He is still on his oxygen to help keep his oxygen saturations up above 93%, and he has been weaning off of it gradually today. He usually desaturates when he has a lot of mucous in his airway and needs to be suctioned, and afterwards is fine again. There really are no other changes. He was up a lot last night and seemed uncomfortable, letting out whimpering sounds, and he was given Tylenol for pain, which seemed to help some. Otherwise he has done all right considering all that he has been through over the last couple of weeks. Heidi, his best buddy, came up to visit him today and met his Grandma. She said she would return tomorrow with a bundle of soda for us to get us through this next week. Tomorrow should be a little more exciting and this roller coaster that we have been on will surely pick up speed again and we will continue our ‘ride’, always unsure of what is to come, but we know it will be there waiting for us without question. We no longer ask, “What else?” because all too often we get an answer. Thanks for all of the prayers and support. We continue to pray for the other PBMTU kids, as there are two others in the PICU with Noah, as well as other Krabbe kids & families, the families who have lost their children to such horrible diseases or complications of them, and for our troops overseas & their families. Thanks so very much and God bless.

Saturday, March 29, 2003 9:17 PM CST

Entry 58---March 29, 2003 Post Transplant Day 38

Today was a long, but quiet day, to follow up a yesterday’s hustle-bustle. Noah had a pretty good night, but still had a few episodes of oxygen desaturation into the 60-70% (normal 93-10%) and turned very dusky, and required some oxygen and stimulation to get him back to a normal level. We learned that he had been given so much pain medication yesterday that this was most likely the reason for these episodes…he was snowed!! We told them not to give him anymore and to let him cry a little to help clear his airway of the mucous and exercise his lungs a little. We don’t want him in pain, but he doesn’t need to be so snowed that it may cause him to need continuous oxygen therapy or to be placed on a ventilator! So during the night and all day today they did not give him this medication and he has begun to open his eyes more. He has been sucking more on a pacifier and moving his legs more, but continues to not move his arms much and opening only his right eye. We hope to see some more positive changes over the next few days. He has not had any more dips in his heart rate and his breathing pattern is more regular, which means that the release of pressure in his head has made a difference. The issue with his abdominal pressure is still a mystery. The abdominal CT results were basically normal except that his liver was slightly enlarged. This was really no surprise to the bone marrow docs, because most of the kids develop this after being on TPN for so long. He had another feeding tube placed that goes down his esophagus and through his stomach into the top part of his intestine and they finally started him on formula tonight. The rate is only at 2cc/hour, meaning that it would take 15 hours for him to get one ounce, but they want to start him at a ‘trickling’ rate to make sure that they give his intestine time to start itself up again slowly, and he will have less chance of getting sick. They are thinking that maybe this will help decrease his abdominal pressure by getting it up and working again, since he has not had any formula since the first part of February. It is worth a try, and besides we want to get him back on formula and eventually try to get him back on a bottle soon. His weight has been relatively stagnant since we got here, even though his photos make him look chubbier, it is the steroids making him appear chubby in his face and belly, but his weight really has not changed all that much.

His WBC count yesterday had dropped to 2.0, which discouraged us, but early this morning it was back up to 2.2 and later up higher to 2.7. So we hope that it will continue to rise and decrease his risk of getting an infection. We learned the results of the ‘chimera’ test, the blood test taken on early Monday morning and sent away to a special lab to check out the percentage of donor cells to his own cells, which indicates his percentage of engraftment. One of the docs covering last weekend told us that he truly expected Noah not to be anything less than 90% donor cells, but Noah’s results show that he is only 46% engrafted (still 54% his own bad cells). So we are going to have to wait for them to run another test within the next couple of weeks to make sure that this number increases and that his own cells don’t overgrow the donor cells, because if this is the case then there is a chance that his transplant is not taking and he may risk needing another one. So we are praying that he continues to not only grow cells, but that he grows DONOR cells! As if we don’t have enough to worry about right now. The weekend should be pretty mellow as we wait for neurosurgery and the bone marrow docs to make their decision as to what to do for Noah. We are hoping that they can find the source of the abdominal problem, solve it and then get the shunt internalized again and get him back onto the bone marrow unit again. Fortunately we have had a much better experience in the PICU this time around, so we don’t feel as upset about being over there, but with him there the bone marrow docs are not in complete charge of his care. Instead they act only as consultants with the PICU docs, because that is not their ‘area’ and this worries us some. We always ask them to refer back to the bone marrow docs before trying to do anything invasive, and if they don’t then we walk over to the PBMTU and tell the docs what is going on. This is probably not the politically correct thing to do, but we have found it to be the safest and best way to make sure Noah is taken care of. So far this has worked well.

We are grateful that Noah’s grandma is here to visit with him and to help us out. She has been sitting with him and comforting him, allowing us to get some much needed rest. We had planned to be outpatient by this point, but obviously that is not the case and won’t be for at least a few weeks. We continue to pray that Noah will make it through all of this, but as we seem to take a step forward, we have to take two steps backwards. It is getting old fast, and we are emotionally, mentally and physically exhausted and wish that this dark cloud we have been under for so long will finally let up and move on so that we can as well. We want to say Thank you once again for all of the prayers and encouragement we have received throughout this journey. Thank you to those of you who have contributed to Noah’s fundraising accounts. We have been using the money to eat each day and plan to use more of it later as we are outpatient to pay for food, diapers, formula, medication prescription co-pays, hospital parking lot fees for our daily visits to Noah’s outpatient appointments at clinic, etc. It all adds up, and we are VERY appreciative for those who have given. God bless you.

Friday, March 28, 2003 4:15 PM CST

Entry 57---March 28, 2003 Post Transplant Day 37

This day got off to a bad start. We got up this morning exhausted after spending all night settling Noah in the PICU. I immediately rushed to get over to the PICU so I could see Noah and hopefully catch the bone marrow rounds. You are not allowed in the PICU from 0700 to 0930 because of PICU staff rounds (something we already disagree with) So I made sure I waited till 0931 to buzz the receptionist. She informed me that I couldn’t see my son because the doctors were rounding. I informed her that I wanted to be there if they were discussing his care. I was told that the doctors would not allow me back. So I continued to buzz her for about five minutes with no luck and then finally came back to the bone marrow unit to see if the nurses over here could get some reply. They too struck out. That was it…I called patient relations. I let them know that they are not doing me a favor by letting me see my son, and we defiantly wont allow him to be touched without our consent. So by 1030 we were finally let in to see him. The nurse told us he was going to CAT scan and that the doctor had ordered a feeding tube placed. Questions… Why? Who ordered what and for what reasons? This has been our major concern, that we won’t be involved in his care plan. With Noah everything has to be trouble shot, one mistake could cause serious damage. We spent about an hour talking to the reps from all the departments today and we let them know what our issues are. Hopefully they realize now that not much gets passed us, and that we are very involved in his care. So what is his care plan? Still working the CAT scans and chemistry. We probably wont have an answer until Monday at the earliest. The most important thing is that he is doing better now that the pressure has been released. We are sure that the medical team will sort it out, hopefully sooner, not later, because until they figure it out we have to stay in the PICU.

Thursday, March 27, 2003 9:58 PM CST

Entry 56---March 27, 2003 Post Transplant Day 36

Noah went to surgery to have his shunt revised today. It has been a long day of “Specialists” who have all had their two cents to add, none of them agreeing with Mom and Dad. They all had their own reasons for the drop in Noah’s heart rate, none of them being because his shunt is not working correctly. Now the jury is in…. Noah’s shunt is working but not draining, basically making it ineffective. So Mom and Dad where correct. You are probably asking yourself, “How is that possible?” Well, for some reason the pressure in Noah’s abdomen, where the shunt drains to, is greater then the pressure in his head. For that reason there is no draining until the pressure in his head is greater then in his abdomen, and his abdomen pressure is 180. To keep him from throwing up, sleeping all the time, going cross eyed, acting like a rag doll, and his heart rate from dropping, the pressure in his head has to be kept at about 70, and there lies the dilemma. So what now? Noah’s shunt was removed and an external was placed again. This lands us back in the PICU, the one place in Duke we hoped never to see again. It is ironic that today we filed an official complaint with the hospital because of the last time we were in the PICU, and now we are back. I wonder how we will be treated this time? I wish I could tell you what is next but unfortunately the doctors aren’t sure what the problem is. We will have to endure the long line of new specialists tomorrow.

Thursday, March 27, 2003 0:42 AM CST

Entry 55---March 26, 2003 Post Transplant Day 35

Sorry it has been a couple days since we have made an entry, but we have been quite busy. Gammers made it okay, and is glad to be here to support us through this rough time. I think she has a better idea of what we are going through in just the day and a half that she has been here. Take a seat and get some coffee because this is a long one. Two nights ago Noah began vomiting more frequently, over ten times in 12 hours, and come to find out it was old blood. Why? Well, on Saturday (3/22) his NG tube was accidentally pulled out by mom and dad. He has this tube only for a medicine with a volume of only 0.2cc (basically 2 drops) and requires about a teaspoon size sterile water flush to make sure it gets into his stomach. The tube was placed after much resistance from mom and dad because one of the docs thought that Noah was aspirating his oral medications. We conceded and gave in to it rather than risk Noah getting sick, however continuing to believe that Noah was swallowing fine and was not aspirating. Well, because his tube was removed, it had to be replaced. It was left out for several hours and Noah began to sound much clearer and his oxygen saturations were higher too, but when his med became due again the tube was replaced and his med & water flush given. Soon after he vomited, and this continued for the next two days with increased frequency. Dad confronted the nurse and inquired if the tube was correctly placed and the nurse said it was. On Monday (3/24) we confronted the doctor about it, saying that it seems to us that something is very wrong with his NG tube, because he is vomiting (old blood) after his meds were given through the tube, but he only said that yes he was concerned about the cause for his vomiting and that it was old blood, but not enough to feel the need to remove his NG tube. So it stayed in and we again felt uneasy about it, but what do we do? As well, Noah began to have heart rate drops into the 70s-80s on late Friday (3/21) and we began to worry about increased pressure in his head again due to possible fluid build up or shunt malfunction, but we were told that it may take a few days for his vital signs to stabilize. His respiratory pattern was irregular and he was having other symptoms similar to those that he exhibited prior to going to the PICU to have his EVD placed over a week ago. By Monday night he was having even more vomiting and very, very pale. He required red blood cells and platelets. He had another head CT scan to check to see if he was having more fluid build up in his brain, and he had a series of x-rays done as well. Finally Tuesday morning we learned that his NG tube was placed incorrectly and was actually up in his esophagus instead of down in his stomach…THIS was the cause of the old blood and the severe vomiting and once again our suspicions were correct. The NG tube was pulled out and we have been giving him his med via mouth without any noted problems. The risk of him aspirating his vomit into his lungs was much worse than the minute amount of medication. As for his CT scan, we think that his newly place VP shunt in his head may be malfunctioning and not draining off the excess spinal fluid as it is supposed to. If this is the case, which it appears to be, then we are back to square one again and he will require ANOTHER surgery!!! The neurosurgery team members have been in and out for the last two days now. Yesterday they decided to “pump” the shunt by pushing on it in a pumping manner to see if maybe there was just an air bubble in it and needed to be pushed out, but it really did nothing. Later in the evening they returned to tap into the shunt reservoir with a needle and aspirate 20 cc (4 teaspoons) of spinal fluid out of it. It was tested and we found a few things out with the labs that were run using the sample. First, the most exciting news was that his protein level had decreased since the spinal tap done 2 weeks after arriving at Duke in January, from 280 down to 220!! The original protein level indicated that myelin had been broken down and its remnants left floating around in his spinal fluid, however as the needed enzyme gets into his brain and starts working to break down those toxins, the protein level will eventually decrease to a normal level (10-40). However, Dr. K is not sure what to think about it because they have not ever checked CSF for protein level this early after transplant (usually checked at 100 days post transplant) and he has had surgery and a shunt placed, etc. But we remain hopeful. As well there was only 1 WBC in it so there is no sign of infection, which is good and his WBC count yesterday was 1.9!!! Also after taking off the fluid his vital signs seemed to stabilize throughout the night. He had no more vomiting until around 0600 this morning, and then his vital signs reverted back to that pattern of dipping low and he also began having oxygen desaturation with it decreasing into the lower 80s% (normal 94-100%). As well the frequency increased throughout the day today. We seemed to spend most of the day again trying to convince them that something needed to be done ASAP. Several trips off the unit down to x-ray and CT scan throughout the day, an EEG, an EKG, a visit by Cardiology, and Neurosurgery a few times. When the shunt was placed in the OR, the pressure setting was placed at 70 mmH2O and if Noah’s intracranial pressure went above this number, then the excess fluid would drain via the shunt into his abdomen to decrease the pressure to keep it at or below 70. BUT since this did not appear to be effectively working for Noah, today at 0800 a neurosurgeon came in to reprogram his shunt at the bedside to 40 mmH2O so that more fluid could drain off if needed. Another x-ray was done to verify that the dial was correctly on this pressure setting, and it was said to be at 40 by the radiologist. However, his vital signs were telling us that this was not helping any as he continued to worsen and required oxygen to maintain adequate oxygen saturation in his blood, but would still have decreases down to low 90s-high 80s % even with the oxygen. Again, it seemed as if we were getting no where and on a few occasions we both lost our cool with a few of the doctors and the nurse practitioner as we found ourselves once again caught in the trap of desperately trying to get them to listen to us as Noah lay there suffering. Why does this happen?????!!!!!!!!! The EEG was not exactly normal due to his Krabbe’s, but showed no signs of seizure activity, which is what they were concerned he might be having. His EKG showed that his heart was ‘electrically’ fine and had a normal heart rhythm. Another neurosurgery resident came in tonight to tap Noah’s shunt AGAIN to relieve some pressure until they could decide on what to do, because it seemed to help him last night. However he brought a pressure gauge to measure his ICP too. We have been told that they are not necessarily the most accurate, but it would be a good indication if there were an increase. Prior to starting we had a Q & A session and inquired about shunt failure once again. He began to go on and on about how this was so very rare and that Noah’s CT showed that there was less fluid in it than the night before when comparing last nights CT with today’s and that he did not think that hydrocephalus was the problem either. Needless to say he doubted us as well, GO FIGURE since that seems to be the consensus! He accessed Noah’s shunt and began to measure the pressure. We asked him what the pressure was, and he said, “Well it is a little higher than I had expected, but not too high”…that did not answer our question. When confronted again he finally stated that Noah’s pressure was reading 110 mmH2O!!! Just imagine what it would have been had they not taken off the 20cc last night! Well, since this pressure reading is well above what the shunt dial is set at (40), then the shunt is NOT functioning correctly!!!! However, the resident wanted to check with radiology to confirm what the shunt was actually set at and talk with his attending first before going any further. We were not getting warm fuzzies that anything would be done about this anytime soon, so we decided to once again call Dr. K, knowing that even though it was 9:00 PM she would not care and would show that she cared by truly listening to us! She reassured us that Noah would not have damage from this because we caught it so early, and that not to worry because first thing in the morning she would contact the neurosurgery attending and let him know that Noah needed his shunt revised tomorrow no matter what. There would be less risk for infection because his WBC count today was 2.5!!!!!!!!! GROW CELLS GROW!!!!!! So presently Noah’s heart rate has stabilized a little more and not dipping as much, but only on occasion. He is not desaturating anymore. He has not vomited since this afternoon. We will see what happens in the morning, but it looks like he will once again be going to surgery. We pray that if this is the case that he will have a smooth surgery without any complications and he will be able to be extubated successfully and sent back to the bone marrow unit and not to PICU after his procedure. We pray that this will permanently solve his ICP and hydrocephalus problems. We know that this will be a learning experience that we can ALL learn from, including the staff, and hopefully if they come across a similar situation in their future they will remember that a shunt malfunction CAN & DOES happen. However, it is frustrating being the ‘lab rat’ so to speak, as we certainly feel that way sometimes. Noah seems to be trying to teach them a lot of new and different things that they have not seen before because they are so rare…Krabbe’s, central line that wormed its way out of one vein and into another up his neck, and now a shunt malfunction. What next? No wait, we don’t want to know!!! We are trying to remain hopeful, but there are days that we feel that we are not going to take him home alive. There are so many dangers that we are still going to face…infection being the big one, in particular respiratory infection from a cold or something. Your support sure helps us, especially during these very scary and trying times. The unit is full again, with a lot of other kids with leukodystrophies (storage diseases) where a certain enzyme is missing that performs a small but important job in the body, such as Hurler’s, San Philipe (don’t know if that is spelled correctly), ALD (Lorenzo’s oil), and Hunter’s disease. There is another Krabbe kid coming soon for transplant as well. These are all kids who have ‘very rare’ diseases, but on this unit they seem quite common. It seems that our own family members who have gone to their own docs and inquired about testing for our nephews have been told that “I wouldn’t really worry about it too much, because it is so very rare and its so unlikely that your kid(s) would have it or be carriers”. This is heard much too often and I bet if it were in their families that they would not be saying that, and they would feel the same fear and sense of urgency that our family members are feeling right now. What a shame. We continue to pray for those families who have lost their children recently, for other Krabbe kids, for the other transplant kids on the unit and the two that are back in the PICU, and for our troops overseas. Thanks so much for your prayers and support.

Monday, March 24, 2003 6:04 PM CST

Entry 54---March 24, 2003 Post Transplant Day 33

Noah has been the king of bodily functions all day with fluids coming out of every possible place. I will let your imagination figure out what the days been like. We have been concerned but the doctor looked at him and didn’t think anything of it. It is not uncommon for kids to get real sick after in graphment. He has been very alert, but also very miserable. We have been preparing for Grandma’s arrival tomorrow. It will be nice to have family here to help. It seems like we have been at this for a year now alone and he’s only six months old. Noah had a WBC of 0.4 yesterday and freaked us out but rebounded with a 1.3 today and an ANC 1040! Great Job Noah! The OT lady came by today and pissed him off, but was also able to get him to suck on his hand. Noah is often disgruntled during physical therapy but we realize it is a necessary evil. We are hopeful that Noah will continue to improve and we can start the nightmare known as out paitent.

Saturday, March 22, 2003 8:12 PM CST

Entry 53---March 22, 2003 Post Transplant Day 31

Noah has had a special friend on the floor since the day we got here. This little girl, three years old, instantly became his best buddy. During her daily exercise walks with her father, she would always stop by our room. She didn’t do much walking, not because she was unable, but because she didn’t like to be set down , so her father continually held her tightly in his arms. She couldn’t come in to visit Noah in his room; she had to wear a mask, as neither child could risk exposure to each other. But her dad would lift her up so she could see her cell mate through the gaps in the pictures we have taped all over the windows. She always looked wide-eyed at “baby Noah” and here visits became like clock work until, finally she won her freedom from the floor by archiving a high enough white count. Unfortunately Debbie and I missed her escape because we were at a Valentines dinner sponsored by the unit, but she returned often during clinic to visit Noah. Besides being Noah’s biggest fan she was unique in another way…. She had endured chemo and transplants three times in a row because her old sick cells kept coming back. This is an incredible achievement for such a little girl, but some how, after the third try, she began to grow donor cells. Her leaving of the floor was like a medical miracle and a testament of her strength and courage. Unfortunately, Noah and his special friend became united again in the PICU when she came down with a bug. Ironically they were both discharged back to the floor on the same day. Because she was ill she couldn’t leave the room to visit Noah, but we went down to check on her. When I visited she was cuddling on the bed with her dad, watching the cartoon network. She looked like any other three year old except for her dark tan from the chemo, no hair, and thick, black, bushy eyebrows from the steroids. The only difference I could see in her was that her look of amazement and curiosity had been replaced by fear and pain. Twelve hours latter the entire staff rushed past our door and down the hall. We didn’t need to ask whose room they were going to, it was obvious. We watched the spectacle unfold from our room through the slits in the blinds. Soon, slowly, several nurses began to walk back down the hall, heads hung low, some of them crying. This child who had been such a miracle and caused such a commotion had left the floor like a whisper. She was now free to visit Noah whenever she wanted without a mask. The next day Noah had a white count of 1.1 and an ANC 686, after 31 days he is officially in graphed.

Friday, March 21, 2003 11:13 AM CST

Entry 52---March 21, 2003 Post Transplant Day 30

What a ride this has been and continues to be! We have had our share of gut wrenching sharp curves, nauseating spirals, awe-inspiring highs and hart stopping lows. We ride forward aware that at any moment the bottom could drop out, plummeting us downward. Eyes wide open, screaming at the tops of our lungs, our harts in our throats and stomachs twisted, we fall into darkness and then stop…. a pause…. The car wretches forward again with a tug, and we start the climb upward once more, as we try to keep our eyes on the horizon. We look out envious at the families in the parking lot, weighed down with balloons and cotton candy, laughing and happy as they load up their car to go home, leaving us in the rear view mirror, glad to be solidly anchored to the ground. Exhausted and pale, our knuckles as white as snow, the ride continues as we come around again, screaming, stop the ride, we want to get off! The feeling of helplessness comes once more as the ride operator refuses to set the brake; he looks at us with pity as we rumble on past the exit. We continue to hold on tight and hope the car does not derail, as we fly head long down the track, unaware of what lies ahead. We look back, and it seems like a dream, the days run in to each other and the ups and downs seem to straighten through the haze and dull hum. We recurrently transition from breath taking speed to disappointing stops, continually reminding ourselves that no one forced us to take this ride. We can only hope, as we come around again, that soon it will end, and we can load up our family to go home, and finally… rest.
Today Noah has a WBC of 0.9 and an ANC of 620. One more day of an ANC over 500 and he is officially in-graphed. Soon after they should draw samples to see what percentage of cells being made are donor cells. At least we are one day closer to stopping this illness and hopefully going home.

Thursday, March 20, 2003 3:53 PM CST

Entry 51---March 20, 2003 Post Transplant Day 29

Noah had a rough night, requiring IV Fentanyl for pain every 2-3 hours, but it still did not seem to allow him to get good rest. His heart rate, respiratory rate and oxygen saturations began to stabilize over the course of the night, which was a relief to see, realizing that he had abnormal rates that had been gone undetected at least since admission. He has been having some problems with nausea and vomiting, which is most likely residual effects of the anesthesia from his surgery yesterday. His VP shunt was placed on the back lower right side of his head, just under the skin, and it runs underneath the skin and all the way down to his abdominal cavity. It is an electronic programmable one that allows for Noah’s doctors to change the pressure settings as needed. When he has an MRI later on down the road, it may cause his settings to be erased and he will need to have them reset immediately by his doctors, but it is really simple and nothing to worry about. He has gauze dressings to the incision where the shunt was placed, where the external drain was removed, and on his abdomen where the drain ends. There is some notable swelling to the back of his head, but he tolerates laying on his right side quite well. The bone marrow doc came in and said that Noah looks good, but is probably having some pain, and they will start another Fentanyl PCA again if needed. They also started up his Ativan medication again today, which is used to help keep him calm and relaxed. Hopefully the combination will allow him to rest comfortably. The plan from here on out is to continue to allow him to engraft and grow those cells. Today his WBC count was 1.2, with an ANC of 696!!!!!!!!!!!!!!!!!!!!!! GROW CELLS GROW NOAH!! KEEP UP THE GREAT WORK BABY BOY!!!! His platelets are low again and he required another infusion today, but otherwise his lab values are looking fine.
Thanks to EVERYONE for the prayers for Noah, the other Krabbe kids and families, and the other PBMTU kids waiting for their cells to grow. We ask that everyone remember our military members overseas and their families that are waiting for their safe return home. We are grateful to our troops for all that they do. HOOAH!! We feel blessed that Noah’s daddy is able to stay with him here at Duke as Noah faces his own battles, and that he does not have to leave to join his peers overseas during this time of war. Thanks to those who have contributed to Noah’s fundraising accounts, we appreciate it more than we could ever express. God bless you.

Wednesday, March 19, 2003 6:11 PM CST

Entry 50---March 19, 2003 Post Transplant Day 28

We awoke today to good news of a WBC count of 0.5, and an ANC count of around 290!!! GROW CELLS GROW, NOAH!!! By around noon we were preparing to take him down to the surgical unit for his shunt placement. We spoke with anesthesia and signed all consents. Noah had been given platelets and packed red blood cells throughout the night and into the morning to get him ready for his procedure. His surgery lasted around 1-2 hours and he was extubated successfully and sent into the recovery unit for observation. He had some x-rays taken there and given some pain medications to help keep him comfortable. Since he was stable and was not intubated/ventilated, he did not have to go back to the PICU and was able to be sent directly back to the PBMTU to his ‘cell’. Thus far he is awake, but drowsy from the anesthesia, and cries occasionally from pain. Overall, from what we can tell, he is doing well. However, it will be a few days before we can really be sure that the shunt is effectively doing its job. He had a programmable electronic shunt in which the pressures can be adjusted as needed with a special electronic device. We were informed that the risk of infection begins to taper off around six months after placement. This is something that we will be watching even when we return home. The neurosurgeon team will be in tomorrow morning to check on him again. We are so pleased that Noah seems to have done so well with all of this. As for the other 3 PBMTU kids that were in the PICU, 2 others returned back to the bone marrow unit today with Noah, but there is still one other that needs to return, and we continue to keep him in our prayers. Thanks to EVERYONE for your wonderful show of support and prayers. We also want to ask that Hunter Kelly (Hunter’s Hope Foundation) be added to the many prayers being said for these kids. He was taken to the PICU two days ago in NY. As well, please remember to include all of our Krabbe kids who have gone through so much, and continue to face such difficult obstacles. God bless you.

Wednesday, March 19, 2003 1:46 AM CST

Entry 49---March 18, 2003 Post Transplant Day 27

Today was another long, eventful day. It began with the neurosurgeon changing his mind again and deciding that he wanted Noah’s WBC count to be no less than 3.0!!!! At this rate it seems that Noah will never get this procedure done. We were left frustrated once again, feeling as if everything was working against us. Where was Dr. K and why had she assured us not to worry, but that Noah would have his shunt placed and be back on PBMTU by no later than Friday of this week???? It seemed to us only to be getting worse and that Noah was only going to be there longer because of the WBC count requirements of the neuro team, and in the meantime his external drain could not be monitored on the PBMTU but only in the PICU. ENOUGH IS ENOUGH, ALREADY!! SOMETHING HAS GOT TO GIVE!!! We feared that it was inevitable that Noah was going to get an infection, and may not be able to fight it off…and may never get out of the PICU. He is doing so well right now, but it doesn’t take much to make him deathly ill. For instance, the first day (Friday) he was in there, we walked over to visit him and found all of the doors to his “isolation” room were wide open. When the nurse was confronted about this and we explained that he was neutropenic and needed to be in tight isolation, she said that the room was too warm and she was really hot in there and sweating. So I guess it didn’t matter that Noah was being exposed to who knows what kind of bacteria, etc., but as long as she was cool and comfortable…that is all that mattered!!! How appalling!!! Not long after that on the night shift, a nurse caring for another patient in the isolation room next door for who knows what, took off her gown, mask & gloves, walked out of their room and into Noah’s isolation room without washing her hands, gowning or gloving and began digging in his supply cart near his bed. What about “universal precautions” where the most basic method of protecting yourself and patients against infectious diseases is handwashing!!!??? We were terrified that his chances for infection were about 100% with this type of behavior. We informed the PBMTU docs the next morning and from then on, the nursing care was much more safe and responsible. It is so hard to stay at his bedside constantly, but we feel like we almost have to, to make sure that Noah is kept safe from these types of problems. Another example was one of Noah’s labs (his glucose) returned as over 400 (normal around 70-110) and the young Resident doctor was wanting to put Insulin in his TPN or start a continuous insulin IV drip!!! If Noah had a glucose blood level of over 400, we are certain he would not have been as alert and stable as he was. As well, the Resident stated that Noah was ‘positive’ for 50cc of fluid and he needed to have a catheter placed to get some urine. They had apparently stopped his scheduled doses of diuretics over the weekend and so instead of trying to give a one-time dose of his diuretic, she wanted to catheterize him. No big deal on a normal kid, but on the PBMTU they don’t catheterize kids due to the risk of bleeding that may occur and most of the kids have such low platelet counts that they may have trouble clotting. Instead, the meds and fluids are manipulated to try and do the trick first. We stopped her from doing both and suggested that she consult the fellow doctor that was on call that night on the PBMTU first before continuing any further. The lab for his glucose was redrawn and came back at 102. Apparently the first lab specimen was drawn and some of his TPN, which contains 15% dextrose (sugar) in it, therefore it gave a false lab result. What do you think would have happened if the Resident had ordered an insulin drip or placed insulin in his TPN…it could have caused some real big problems!! As for the other issue, she conceded and gave him a dose of diuretic first and sure enough, Noah put out over 100cc within 15-20 minutes. As like any parent, we worry having to place Noah’s life in just anyone’s hands, on top of worrying about his current health status & the progression of the Krabbe’s, etc. We are very tired, and what emotions we have left are constantly being pulled left to right and up and down. It doesn’t take much to get us worked up when it comes to our little boy nowadays. As all of the other parents who have gone and are currently going through similar situations are aware of, it is so hard to ever put into words just how exhausting this is with the continuous roller coaster of emotions, hope, frustration, devastation, grief, etc.
HOWEVER…by just after noon we were given some much lighter news. Apparently the neurosurgeon had a change of heart after talking to Dr. Kurtzberg and came in to tell us that Noah’s shunt would be placed tomorrow around noon time. He stated that Noah would be the first patient that he has performed surgery on with basically no WBC count, but that in the end it was agreed upon by all of them that the risk for infection may be less by placing the internal shunt rather than leaving the external drain in and waiting for a specific WBC count. She said that she would take care of it and she did. So Noah will have his shunt placed tomorrow, and we pray that all goes well. He will return to the PICU intubated on a ventilator most likely, but should hopefully not require this for too long. Once he is off of the life support and stable, he will be transferred back over to PBMTU, THANK GOD FOR THAT!!!!! As well, we were told that this most likely won’t interfere with his engraftment process and that if he continues to grow his cells, he may be out of here and be outpatient status in a couple of weeks. That would be awesome, but just getting him back out of the PICU and in his room is great. Noah’s WBC count today was 0.4 and his ANC was 264!!! GROW CELLS GROW NOAH!! Thanks to all of you cell dancers doing the cell dance! We pray that Noah does well tomorrow and that he makes it through this without getting an infection. He remains stable and pretty much the same for the last two days. At times he is fussier than he has been lately, but nothing like he was before his diagnosis of Krabbe’s! We have no problems handling him, he is so easy to deal with now. They will give him meds to help calm and relax him as needed, but overall he has done so well.

We had the privilege of meeting Matthew, another Krabbe kid who was the second Krabbe kid ever transplanted at Duke a few years ago, who is here for treatment of an unrelated problem. What a cutie! God bless them for their strength, as 3 of their 6 (or 5?) children were born with this disease, one passed before transplant was an option, and the other two were transplanted at Duke and are doing well. We wish him well and pray for a speedy recovery. We also want to mention that there are 3 other PBMTU kids in the PICU besides Noah, and we ask that you will remember them in your prayers as well. It is hard to see not only our own child going over to PICU, but to have some of the other kids over there, with whom we have become so attached to along with their parents, that it adds so much more emotional weight to our burden. We pray that they will be back over to PBMTU real soon!

One last correction on the smiles quilt address. Apparently it is www.smilequilt.com (without the ‘s’) and we were mistaken. Just the same, please check out his smile quilt via the web page. It is listed under page five of the quilt index. Again, thanks to everyone for all of the prayers. We DO appreciate it and are blessed to have so much support. It does make a difference. God bless.

Tuesday, March 18, 2003 2:13 AM CST

Entry 48---March 17, 2003 Post Transplant Day 26

HAPPY ST. PATTY’S DAY, GROW CELLS GROW!!! Noah remains in the PICU with no significant changes, which is good. However, we want to get him back to his room on PBMTU ASAP. The story has not really changed much since yesterday. Noah’s WBC count today was 0.3 again and his ANC was 126!! GREAT!! However, the neurosurgeon team has now decided that they want his WBC count to be between 2.0-3.0!!! Meanwhile, he will need his current EVD drain replaced in a few days, because they are only good for 7-10 days and the longer it is left in, the risk for infection becomes greater. As mentioned previously, the overall risk for infection with the external drain (EVD) is 1 out of 3 chance, but that is for “normal kids”, so because he is neutropenic (no WBCs), then what is his chance? 3 out of 3?!! AND if he gets an infection, although localized to his head, even if he were to grow some WBC and his count would increase it would no longer matter, as they will not surgically put in a permanent shunt until he is infection free. However, he will not likely have a significant WBC count for some time, so in the meantime he will be at a HUGE risk for getting an infection while we wait for his count to rise…and in the end it would not matter. We can see the position that the neurosurgeon team has, but Noah is not the typical patient, and overall, the risk of infection for surgically placing the internal shunt is less because it is not exposed to the outside environment…but if he were to get an infection it would not necessarily be localized to just his head, but would risk running the entire length of the shunt (from his head down to his abdominal cavity where the shunt empties) and could potentially be a much more serious infection. Another issue involved with this situation is the possibility that Noah might have to be intubated with every change of the EVD and then later the surgical placement of the permanent shunt, which poses certain risks in and of itself, and of course we don’t want him to go through this anymore than he absolutely have to. If they would agree to put in the shunt then he should only require intubation one more time, but if they refuse to do it now, then he may require it for every EVD drain change until his WBC count is sufficient for them. Basically it is all about weighing the options and deciding which will be of more benefit & less risk, but it won’t really matter what we think…we can only argue our opinion, but we cannot make them put the shunt in and at this point in time he at least needs something in place to drain off the excess spinal fluid, whether it is the temporary or permanent drain. We are hoping that Dr. Kurtzberg is still battling it out with them, but we have not yet spoken with her since last Friday. We plan on waiting until later tomorrow afternoon to call her and find out what she has been doing behind the scenes. In the end, whatever she feels is best for Noah is what we will support, because she knows what these Krabbe kids need and cares so much about them. As always though, we will not hesitate to remain a thorn in the doctors’ sides and trouble shoot them until we feel satisfied that Noah is completely out of harm’s way and they are doing what we think is absolutely the best for his sake. We are VERY appreciative of those particular doctors who did not only listen to what we had to say, but decided to investigate further to validate whether or not these complaints were parental paranoia or actually legitimate. As we have heard so often and personally relate with, it makes such a difference to be taken seriously as a parent when voicing concerns about their child, who they know inside and out. There is nothing more frustrating then being made to feel paranoid or as if we don’t know what we are talking about just because we don’t have the “MD” behind our names. So again, we appreciate those doctors who have taken our complaints and concerns about Noah seriously. Special thanks to all of the Krabbe family members that have taken time out to visit Noah’s page and sign his guest book. We appreciate all of the AWESOME support we have received…and you ALL know who you are!! Thanks SO MUCH!!! Please check out Noah’s smiles quilt at www.smilesquilt.com under page 5 in the quilt index. Thanks to Reese’s family for sharing it with us. God bless.

Sunday, March 16, 2003 1:43 PM CST

Entry 47---March 16, 2003 Post Transplant Day 25

HAPPY 6 MONTH BIRTHDAY NOAH!!!! GROW CELLS GROW!!! Oh what an emotional roller coaster we have been on these last few days! To catch everyone up, on Friday afternoon Noah’s IV sedation/pain medications were turned off and he was allowed to gradually wake up while still on the ventilator. As well, he was weaned on his ventilator settings throughout Thursday night/Friday morning until he was basically on “room air” level of oxygen. By late Friday he was stirring around a lot more, and grunting & moaning in discomfort because he was awake and intubated! What an icky feeling that must be! Due to an emergency in the PICU with another patient, he was not extubated until around 0400 Saturday morning!! Bless his little heart, he had to go a VERY long time lying there awake with the breathing tube down, but again as we have said throughout all of this, he was such a trooper and was so good about it all. After he was extubated, he sounded really raspy again, as when he had mucocitis really bad, and he again had large amounts of mucous in his airway. He continues to have the EVD drain coming out of his head to drain off excess spinal fluid when the ICP (intracranial pressure) goes over the normal pressure range, which is somewhere around 5. When he begins to cry, then his ICP in his head increases to 15-20, and his EVD begins draining a lot of spinal fluid off; it is amazing to see it all work. The physics of the entire drain/pressure system is basically the same as siphoning out our fish tank water and draining it into a bucket; basically, it all has to do with gravity. As of now, Noah seems to be improving neurologically…he is more alert, with both eyes wide open and he has better tone and mobility in his head & limbs. In general, he is beginning to look like the little boy we carried in here over a month ago. The last that we had heard from the neurosurgeon team, they had wanted Noah to have a WBC count of 1.0, but would settle for a neutrophil count (neutrophils are a specific type of white blood cell that are ‘warriors’ and actually fight off infection) greater than 500 before they would consider taking him to surgery to place a permanent internal VP shunt. We could be waiting a couple more weeks for his counts to rise, and meanwhile Noah could get an infection from having that external drain in for too long…then for sure he would not be able to go to surgery to have this procedure done. He would be “safer” in his hepa-filtered room back on the PBMTU amongst other immunocompromised kids, but the staff is not trained to care for his EVD drain/ICP monitoring. However, dad tried to convince them that mom could care for it, but it didn’t work, so Noah is still in the PICU until he can get a shunt placed. This is what Dr. Kurtzberg told us not to worry about, but that she would battle it out with the surgeons and get the shunt placed ASAP. So we are waiting to see what happens tomorrow, and later this week. His WBC count went up to 0.3 on Friday night, back down to 0.2 yesterday, and is now 0.3 today! GROW CELLS GROW!! He did have a neutrophil count of 96 today!!! The attending on call this weekend for the bone marrow unit said that Noah looks GREAT compared to before and he is certain that Noah will have a permanent shunt placed sometime this week. Their plan is to get a shunt placed and get him back over on the bone marrow unit ASAP. Noah is definitely engrafting now, which is great to see, but we are anxious to have this surgery done and scared about the risk of infection that is involved if it is not done soon enough. Again, we will have to try and be patient, watch and wait to see what happens tomorrow. Thanks to everyone, including our Krabbe family, who has offered such wonderful support. We are so blessed to have so many behind throughout all of this, and it is hard to feel so alone with all of you offering such great encouragement. God bless.

Friday, March 14, 2003 3:57 PM CST

Entry 46---March 14, 2003 Post Transplant Day 23

Last night around 2130, Noah was wheeled over to the PICU. His platelets were checked prior to leaving and his labs came back showing a count of only 19 (very low). He had to receive platelets first and foremost before anything else could be started, to lessen the risk of bleeding out. The magic number that the neurosurgeons were looking for was 100, but they would settle for no less than 70. A unit of platelets was hung to infuse and he was wheeled into is isolation room in the PICU around 2200. We had to leave him there while they intubated him and placed him on a ventilator, took a chest x-ray to confirm that the tube was in the right spot for optimal ventilation, and attempted to insert an arterial line, which would be used to directly and continuously measure his arterial blood pressure…but they were not able to get it into his tiny artery, so they decided just to use the old blood pressure cuff to monitor his pressures. We went back over to Noah’s room and made some phone calls and tried to ‘relax’. We were not called until over 3 hours had passed. We were able to return to him around 0200 this morning. He definitely did not look great with the breathing tube in place, but he did appear comfortable and relaxed. We were continuously watching his monitors and his heart rate did not fluctuate at all, it was consistent, low in the 90s, but consistent. He was on sedation and pain medication, which kept him calm and comfortable. We held his little hands in our own and let him know that we were there. He was so very cold, but the room temperature was to blame. Gradually the room became a little warmer after messing with the thermostat, and meanwhile we piled on receiving blankets to try and keep him cozy. Around 0230 or so, one of the neurosurgeons came by and discussed the plan for Noah. He said that despite Noah’s lack of immune system, he needs to have some sort of drain placed to relieve the pressure, or damage would ensue. The best method for accomplishing this at this time would be to place an external ventricular drain (not sure if that is the exact term, but for short it is termed EVD); and this is a small tube place through the skin, skull and brain tissue into one of the ventricles---which basically are small cavities within the brain that contain some of the cerebral spinal fluid (CSF)---allowing any over fill of fluid to be drained by gravity into a bag hanging at the bedside. The tubing is connected to a ‘transducer’, which is able to read the intracranial pressure (ICP) within Noah’s head and transduce it onto the monitor, where all of his other body functions are monitored. We were told that there is a 1 out of 3 chance that this drain would get infected, and is usually seen around 1 week after having a drain placed. This is of BIG concern, since Noah has no immune system. (His WBC count was back down to 0.1 today) He also has a small chance of bleeding, but this is of minimal concern compared to the infection risk. We had no other choice but to go through with it at this time, and so we left Noah around 0300 to allow the doctor to work his magic. We were called back over to the PICU around 0500 to find Noah doing well and stable, but another nasty tube in, this one looking worse than the others since it is coming out of the top of his head. We were shocked to hear that there was very little CSF that came out and that the drain was actually clamped and Noah was maintaining constant ICP. There was some concern that the drain may not be in the correct spot, but they don’t believe this is the case. A couple of reasons for seeing no immediate gush of CSF as expected could be that it will come slowly over the next several hours and we need to just be patient. As well, he was ventilated for a few hours prior to him having the drain placed, so by doing so, the doctors can control his breathing completely. This means that they can manipulate his ventilator settings, thus manipulating the carbon dioxide levels that he is allowed to breathe out. Elevated levels of carbon dioxide in the blood vessels of the brain cause the vessels to constrict, thus altering the ICP in his brain well before the drain was introduced. We don’t know for sure, and we are still trying to understand it all, but it makes better sense when they explain it. However, we took a few photos while we were there, may be a little disturbing to some, but it gives a good picture of what this little guy is having to endure. It is reality to us, and we wanted to be able to share with everyone, both the good and the bad parts about this entire experience. Transplanting these kids can save their lives in the long run, but they have to endure so much to get there and a lot of it can be life threatening too. We left Noah around 0600 to go catch some breakfast and then a nap while morning rounds would be going on between the doctors and nurses. Later in the morning we spoke with Dr. Kurtzberg to see what she was thinking, and she said that there was no question about it, Noah needs a permanent VP shunt placed, and the sooner the better. She said that there have been other Krabbe kids that have had them and she has been able to learn from them about the best approach to take, and she feels that it is best just to put it in ASAP. She also mentioned that we caught it early and she did not feel that any damage was caused on top of what the Krabbe’s has done, and that he would improve greatly by having one placed. As well, she said that we may hear thirty or more different opinions about what to do, how to do it, and when, but to listen to her and the other PBMTU attending doctors. We made it back over to the PICU to visit Noah around noon and he looked good. The PICU resident came in and mentioned that the sedation/pain meds had been turned off, and they were in the process of weaning him off of the ventilator. Once he begins to wake up and to breathe more on his own, then they will consider extubating him. Then he can be transferred back over to his room with his EVD, and our nurses could monitor it. This was great to hear, because the neurosurgeon earlier in the morning said that Noah might likely be there for at least a week. The attending neurosurgeon came in to discuss their plan, and stated that they did not want to take him in to surgery without a WBC count. However, we may not see a WBC count for a couple more weeks, who knows? Leaving that EVD in for a week or more to wait for a WBC count could lead to infection just as the risk of taking him to the operating room to have a permanent shunt placed. We have no idea what is worse. He has been on antibiotics for a couple of weeks now, so he is loaded up with them in case he should go to have surgery and this brings us some solace. Dr. Kurtzberg said to leave this up to her and she would get back in touch with us. At this point, the PBMTU team and the neurosurgeons are battling it out over what should be done and when. Noah is beginning to open his eyes and he is trying to suck on his breathing tube. He is still not breathing over the set rate on the ventilator, which means that he is not initiating breaths on his own yet, but to see his eyes open is such a relief. We hope to see him better soon. Thanks for all of the added prayers for him. We truly appreciate all of you for your continued support.

Thursday, March 13, 2003 7:49 PM CST

Entry 45------ March 13, 2003 Post Transplant Day 22

There have been some questions as to who writes the journal entries. The answer is both of us do. The best way to tell who is writing is to look at the style and content. If the entry has a lot of medical terminology in it Dad didn’t write it, that’s all nurse Debbie. Yesterdays entry I started, Debbie interjected the technical terms into it and ended it. So it is a team effort that depends on who has the need to express them selves on a particular day. We try to be honest, and informative, realizing the web page is the daily update for many friends and family. It also gives us a chance to share with everyone who has been following Noah’s journey the trials and tribulations we are experiencing.
Noah’s white blood cell count (WBC) was 0.2 today, up after two days of 0.1 so that was good news. His rash has gotten much worse, this is a sign of cells coming in and could also be from the reduction in his graph versus host medication FK506. The medication reduction has straightened up his eyes, so we can live with a little bit more rash. He still has a lot of mucus but again that is a sign of cells coming in.

As our day has progressed, it has become worse and worse. Dad went back to the hotel to rest and mom stayed with Noah. Heidi came up to see Noah and during her visit we noticed Noah’s heart rate would drop to the 60s-70s and return up to his baseline around 120s within a short time. He has done this for about two weeks now and mom brought it to the attention of one of the attending doctors out of the five that cover this unit, and he reported via a nurse that there was nothing to really be concerned about since his heart rate would return to his normal rate so quickly. He believed that when Noah would hold his breath that his heart rate would drop some, but returned to its normal rate when he would breathe again. We just continued to watch with some concern, but feeling that we were probably just overreacting and being typical parents. Well, a few days ago when Noah’s eye issues arose we again addressed the issue of hydrocephaly with another attending that was covering the floor this week. Mom has been concerned about this for some time since Noah’s fontanels, the soft spot on top of a baby’s head, closed a little earlier than when most babies do. The fontanels is often used to determine if a baby is well hydrated, dehydrated or over hydrated by its appearance and by feeling it. If it is sunken in then the baby might likely be dehydrated and need fluids, if it is bulging then the baby might likely have too much fluid and need it removed. However, when a fontanels is closed, there is no way to know except by looking for other symptoms such as elevated blood pressure, decrease in heart rate, irritability or lethargy, visual disturbances. Well A LOT of the medications that Noah is on have the same exact side effects; steroids and some of the chemotherapy meds can cause an increase in his blood pressure, the sedating meds can relax him and cause his heart rate to decrease and also cause him to be sleepy and seem lethargic, his Krabbe’s and the high dose steroids can cause irritability. So how do we truly know that he does not have anything wrong? A CT Scan. During some of his drops in his heart rate, his oxygen saturation percentage dropped down to the 70s% (out of 100%) and his face/lips turned dark. He would return to the normal range of 94-98% within several seconds, but this was something that he had not done previously with his drops in heart rate. The Nurse practitioner came in to assess him and suggested at least talking with the doctor again about it. He came in and mentioned that at this point, the next thing to do would be a CT Scan. It seemed that he was not completely convinced that it would show anything different than the one done during our first week here at Duke, but at least it would rule out hydrocephaly. We were pleased that at least we could get it done and know for sure, but as our intuition has proved in the past, we would be proven correct. Sure enough, Noah has hydrocephalus---too much fluid around his brain---and if left alone, the pressure would build up and could kill him. Our next thought is how long has he had this and has any damage already been done to his brain? The Krabbe’s has done enough, and he doesn’t need anything else causing him brain damage. This kid just can’t get a break! The Neurosurgeon will be in shortly to let us know their plan of action, but he will be taken in to surgery tonight. “It is not emergent, but it is urgent” is what the fellow doctor who is on staff tonight told us, and said that Noah’s platelets are too low at this moment for him to have surgery, because he could bleed to death. So they have to give him an extra large unit of platelets before they take him to surgery. So what do we do about the fact that he has next to no immune system? Pray, and pray hard that he gets no infection from the surgery. He has so much working against him right now, and it seems like we are constantly being bombarded by something else. Once he is operated on then he will be in the PICU (Pediatric Intensive Care Unit) for unknown amount of time. We ask that you will continue to remember him in your prayers. He has fought so hard for his little life and always seems to be knocked down, but this little guy always seems to fight harder. We are not sure what will unfold over the next couple of days, but we will do our best to try and update the web page. Thank you all for your continued support and prayers, they are much appreciated and definitely needed at this time.

Wednesday, March 12, 2003 8:59 PM CST

Entry 44------ March 12, 2003 Post Transplant Day 21

Noah has had a WBC count of 0.1 for the last two days now. This is incredibly frustrating for both of us. Since the day he was diagnosed it seems like we are in a race against time. Every day he goes without his new blood factory doing its job is another day of possible damage to his brain. Of course there is also the continued worry of infection. So this week has been one of a lot of anxiety. Yesterday Noah began to have problems with his eyes. He would be looking at you with one eye and the other would drift off. This scares us to death because of what could be causing it. The doctor came and looked at him and told us what the possibilities were. He said it could be from the medications, Noah’s level of his graft versus host medication level in his blood, which was at 34 and they like to keep it at 15 to 25. A side effect of too much of this medication is eye problems. So the doctor skipped his dose last night to bring his levels down. Another cause could be fluid building up in the brain (hydrocephaly); this is from the illness and will require an operation (insertion of a shunt) to relieve the pressure. The last cause could be that the disease has damaged the area of his brain that controls his eyes and could mean brain stem damage and blindness. We are praying it is the medication and have been watching him closely today. Hopefully we will see some improvement soon. Noah’s mucous production has picked up over the night, but it is clear and hopefully a sign of cell production. He has been getting a lot of suction to keep his airway clear; Mom is the master at suction while I am a little bit more conservative. Kelly came by and it was so good to see her and talk with her a bit about things that have been going on. She let us escape from prison for a while and get out for some fresh air for a couple of hours. Tomorrow Heidi will be by to do the same; we are getting spoiled! It is great to have them here to help us out. Thanks to Kelly and Heidi!!! GROW CELLS GROW NOAH; IT IS TIME TO GET DOWN TO BUSINESS!!!

Tuesday, March 11, 2003 7:31 PM CST

Entry 43------ March 11, 2003 Post Transplant Day 20

We received one of Noah’s evaluations the other day and it painted a bleak prospect for Noah’s future. After reading it I felt it was important to address some of our thought process for going forward with the transplant. From the day a child is born he is evaluated and measured. He is placed on a numbered line with statistical data determining the normal ranges for his age. The numbers assigned to the data collected determine whether or not your child is above, below, or at the norms, as established through scientific study, and population sampling.
Noah falls short on many of these curves, he is not normal, and he never will be. In the report it said that, “Noah’s quality of life would not benefit from the transplant process because of the loss of his motor skills.” We were told we would have to make an ethical decision. So, what did we have to consider in the ethical decision making process, what were our choices? We could have decided to just go home. Knowing that within a year our son’s health would bit by bit digress until he died. Trying to medicate him to keep him comfortable while watching him slowly slip away. There was no doubt how that option would pan out. The other choice was a stem cell transplant. There were no guarantees of how this would end up. It is a relatively new procedure and not much historical data exists to predict the outcome. At best case Noah could re-myelinate and gain back some or all of his lost motor function. At worst case he could be a total vegetable, confined to a wheel chair, relying on tubes for most of his bodily functions. The middle of the road outcome would be for him to be confined to a wheel chair, able to use his hands, and capable of speech but with some difficulty.
So, our choices were pretty clear-cut, death or handicapped. We sat in the hotel room holding each other and crying as we discussed our options, realizing the impact our decision would have on our entire family and our future. We discussed with great sorrow and some guilt packing it up and going home. Several concerns came to mind. How could we come so far and give up? Could we quit, never knowing what our son was capable of? What impact would not going forward have on our family, could they ever understand? Would they hate us for not having the courage to try? Could they ever comprehend the difficulties and sacrifices of raising a child with special needs? Did we fully realize ourselves what we were getting into? We thought about Noah and how he would feel, possibly bound to a wheel chair for life. Would he think we were selfish for not letting nature run its course? Could he forgive us if the outcome was devastating? The questions seemed to ramble on with no answers. After agonizing for hours we chose to try to save our son. We would hope for the best and deal with the worst.
Of all the unknown variables in the decision making process the one that swayed us the most to continue was love. The evaluation talked about him having a diminished quality of life. The things that cold be measured would fall far short of societies norms, but there was one thing science could not measure and that was love. No matter how bad the outcome, Noah would be loved, and would know it. We were sure we could make him happy, happy because there would never be a doubt that he was our son and that we loved him. There are plenty of children in this world graced with physical and mental abilities that are not truly loved by their parents. Science would measure them as gifted, but I would have to ague that they were lacking, missing out on something they will probably spend their lives searching for. Noah will never have to look any farther then his parents, there will never be a question in his mind of weather not he is loved. When he grasps your finger our looks in your eyes you can tell he knows this is true.
Looking back, what was the ethical question we had to answer? Was it that death would be better than a life handicapped? Science would answer that question with a yes. It would ague that natural selection dictates he dies. His genetic code is flawed; he was not meant to live or reproduce. He is a burden to a society he can never contribute too. The costs outweigh the benefits. To us that answer, is the unethical choice, it sells the human sprit short and discounts love. So we have chosen to use science to save him, and will let God decide the rest. That is what we think is the ethical thing to do.

Monday, March 10, 2003 7:53 PM CST

Entry 42------ March 10, 2003 Post Transplant Day 19

Today was a difficult day for us, not because Noah is doing poorly, on the contrary he is doing very well, but because one of the children on the floor passed away today. It is scary to think your child could go so far (He was in graphed and at around 100 days on his way to going home when he got sick) and then succumb to something only visible in a microscope. There is only so much you can do to protect your child from this hazard and you can’t see it to fight it. We have worked so hard to get Noah here, spent months with no sleep, shed gallons of tears, gone broke, and developed calluses on our knees from praying, and all our sacrifice could be wiped away by a minute virus, a strand of DNA. Call us paranoid but this scares us to death, especially since we are aware that another family’s son was also brought to the PICU and is in the similar circumstances. Two families out of ten have been taken to the PICU, it’s depressing to walk past the empty rooms, and when you meet the parents in the hall your are left speechless, their sorrow outwardly visible without words. It is so hard as parents, the ones, who provide and protect your children, to be left so helpless. At times it makes you want to cry out, why, is there no justice? So we continue to take it one day at a time and hope for the best. Please add all the families up here to your prayers.
Noah had a WBC of 0.2 today, unchanged from yesterday, but at least not going down. He required infusion of platelets and red blood cells today, but that was expected. His rash is approving so the doctor lowered his steroid dose. This should help him sleep better tonight because the last few evenings the higher doses have made him really restless. Debbie and I are exhausted from pulling alternating night shifts with him and are hoping for a break. His breathing and mucus have really cleared up and he sounds much better with his O2 stats sitting at around 96% to 97%. We hope soon to see 98% to 99% stats, which is what he had prior to chemo. He was very awake today with wide eyes and following mom and dad as they talk to him. This is great to see I only wish we could of got him to smile. Mom and Dad were able to get away from cellblock 5205 on the work release program, when a volunteer from rock a baby stopped by and sprung us. We used our hour to pick up our mail and get our things together to pay bill and do taxes. We are thankful for all the support and prayers and look forward to a higher WBC count tomorrow, GROW CELLS, GROW!!!!

Sunday, March 9, 2003 9:51 PM CST

Entry 41---March 9, 2003 Post Transplant Day 18

Noah has done well this weekend, his rash has begun to diminish, and his mucocitis has begun to heal. He was given a total of 4 high doses of steroid and at first it did not seem to help, but the first day after finishing this medication, his rash began lightening up. He was switched to another type of steroid medication, so actually we aren’t sure which one is doing the trick, but who cares…he looks great!! He has still been getting his aerosol treatments, although he does not seem to have any issues in his lungs and it is really only his upper airway that is affected. However, the treatments stimulate coughing, and with frequent suctioning he seems to get rid of a lot mucous that is stuck in his airway. He is sounding so much better when he breathes. His fading rash means that we should start to see an increase in his white cell count soon. It dropped down to 0.1 yesterday, but it went back up to 0.2 today. So we are back where we were two days ago, but hoping that his count will begin to continually rise soon. The docs came in today for a very brief visit and said that all looks good and we should see his count rise sometime soon. So we are eagerly awaiting tomorrow’s lab results. Noah is holding strong and we are so proud of him.

We would like to ask for your prayers for another sweet baby boy that is almost to the end of his “100 day” wait to go home after his transplant, but became VERY ill after catching a common cold bug while outpatient and is now fighting desperately for his life in the PICU here at Duke. If he does not improve over night, then his parents are considering the most difficult decision of their lives to withdraw life support from their son. We pray that God will watch over this entire family during this extremely difficult time. We pray that his parents will not have to make this decision in the morning, but if it comes down to this, that they will be given some inner peace. We cannot even begin to imagine their pain and grief, but we recognize the fear, desperation, and hopelessness that is felt when the threat of losing your child is staring you straight in the face. If we receive their permission, we may post his ‘caringbridge’ web page address in our next journal. So please remember them in your thoughts and prayers, because they are really hurting right now. Thank you and God bless.

Friday, March 7, 2003 10:11 PM CST

Entry 40---March 7, 2003 Post Transplant Day 16

Noah was up almost all night long due to the high dose steroid therapy he is getting for his rash. The normal dose for a baby his size is 6mg and he is getting 150mg with each dose. A side effect, which we were quick to pick up on, is irritability and discomfort. Unfortunately, the doses were timed to be given at 0100 and 1300, and his severe irritability would begin about an hour after the dose was given and last for about 4-5 hours straight. It would have been nice if they were timed at 0800 and 2000, that way we could at least get some sleep. He only has one more dose to be given at 0100 in the morning, so hopefully it will be our last night like this for a long time. Dr. Kurtzberg said that this med does not seem to really be helping him. His rash is still heavily present and the creams don’t seem to be doing much either. They have changed his med over to another called FK506, and this will hopefully do the trick. Bottom line is that his donor cells are coming in and are causing some problems with Noah’s skin but nothing else, so that is a good thing. Noah’s donor cells are setting up shop and making white blood cells; if Noah’s old blood producing marrow was coming back we would see a white count with no rash, that would be a bad thing. So for now we can only wait and watch. His white cell count today was 0.2 a sign that his marrow is up and running, just not efficiently enough to sustain and protect him. We can only wait and watch and try our best to protect him from infection. Grow Cells Grow.

Thursday, March 6, 2003 6:47 PM CST

Entry 39---March 6, 2003 Post Transplant Day 15

Noah seems to have a habit of getting the excitement started very early in the morning. Over the night there was some concern about his fluid balance, and whether or not he had too much in his system. So, they have been weighing him 3-4 times a day to assess changes. He was 6.9kg at the beginning of the week, was 7.2kg yesterday and was 7.3 during the night. He was given some diuretics and lost a lot of fluid in his diapers, and was back down to 7.2kg early this afternoon. However, with the diuretics, he also lost a lot more of his potassium and some of his sodium in his blood. So he has required some potassium IV boluses again to increase his blood level. The doctor said that too low potassium is not as large a risk to the heart as too high potassium levels. So we are trying not to worry so much about this issue anymore. Some of the reason for the low electrolyte levels was due to an anti-fungal medication that is being used for post-transplant prophylaxis. This medication is hard on the kidneys and causes potassium to be washed out quickly. His morning lab results reflected these side effects and his creatinine level was elevated as well, which indicates some stress on the kidney function. His medication was changed to one that was less harsh on the kidneys and would be a little more tolerated by his system. His platelets were again low and he received another unit of platelets this morning. However, the value we are most eager to find out about was the same again today. His white blood cell count remains at 0.1 for the third or fourth (?) day in a row. Maybe tomorrow we will see a rise.
There was some concern by the oncoming nurse this morning that Noah seemed to be exhibiting some signs of respiratory distress, such as increased work of breathing, harsh breath sounds, and his monitor was reading a lower oxygen saturation percentage (a measurement used for assessing the amount of oxygen in the blood) than he had normally been demonstrating. We are pleased for her concern, but weren’t sure that he was really any ‘worse’ than he has been since his mucocitis set in. To be on the safe side, Noah’s nurse practitioner (NP) Bridget came in to check him out and decided that maybe trying an Albuterol aerosol breathing treatment would help him. These treatments are commonly used for asthma patients to help open up ‘tight’ airways, and both the nurse and the NP thought that Noah’s airways were tight and his ability to move air in and out restricted. However, he continues to still have inflammation and a lot of mucous in his throat and upper airway, which creates a lot of noise and resonance. So when listening to his chest, he sounds noisy everywhere and it is difficult to determine just what his actual lungs sound like. After his second treatment in the afternoon, the respiratory therapist said that he felt Noah’s lungs were actually relatively clear, and that Noah was likely having some tracheal swelling, which was causing some noise, along with the increased mucous in his throat. He suggested that the Albuterol treatments be discontinued, because they would not really be helpful to Noah. Instead, Noah would just need a little time to overcome his mucocitis and to just wait for the tracheal swelling to diminish. Noah sounds a lot better to us than he did a week ago, so we are not as worried with how he is doing in this area.
His rash reached its peak today, with his cheeks both very bright red. It became more pronounced on his trunk, arms and legs, so to be on the safe side, his IV steroids were increased to help treat it better than what the creams were supposed to be doing. They didn’t seem to do anything for him, so hopefully this will help relieve some of his rash. We aren’t so sure that it really bothers him, but it looks absolutely miserable. The doctors are pretty sure that it is an engraftment rash and not a graft vs. host, but sometimes they appear as pretty much the same in the beginning. So we’ll continue to monitor it and hopefully we will have some higher white cell counts soon.
We have received a few care packages from home in Hawaii, and have shared some of the goodies with the staff. They seem to really enjoy the dried pineapple and macadamia nuts. It was so nice to receive something to remind us of home. Although we have only been here about 8 weeks, it seems like it has been much longer. We have such a long way to go before we can return with Noah, and we are eager to take him back home with us as soon as we can. It will be great to take him in to see the doctors that helped get him here and to begin an education crusade to increase the awareness within the hospitals there. It is so important to us that no one have to experience the frustration and desperation that we felt trying to get someone to listen, and all the while Noah continuously deteriorating and losing precious time of his little life. If we can share anything about our experience, it would be to listen to your gut instinct, a.k.a. parent’s intuition, and to be consistently aggressive. If nothing is wrong, then there is no harm done and you can sleep at night having some peace of mind, but if something turns up, then as it was in our case, we had the opportunity to try and save our baby’s life before it was too late. GROW CELLS GROW NOAH!!!

Wednesday, March 5, 2003 8:53 PM CST

Entry 38---March 5, 2003 Post Transplant Day 14/ (2 weeks)

Well, Noah got things going a little early this morning. He received a unit of packed red blood cells for a low hemoglobin level at around 0430. He ran another fever last night for a short time and again did not require any medication to treat it. Due to him having a couple fevers, he had a chest x-ray done this morning to check out his lungs. He continues to have a rash all over his body, but it has become most remarkable on his face, and his cheeks are bright red now. We have some special steroidal creams/ointments to place on his rash several times throughout the day. We are thinking, and hoping, that this rash is a sign that Noah is engrafting his stem cells. GROW CELLS GROW!! But on the more negative side, rashes and fevers are also some of the signs of ‘graft vs. host disease’, when the donated cells attack the recipient’s body. We will be closely watching to see if Noah’s white blood cells rise in the next few days after having had this rash for a couple of days now. GROW CELLS GROW!!!
During the rounds with the doctor today, he felt fine with where Noah is at, and had no other significant concerns at this time. He stated that we might see some white cells popping up soon. GROW CELLS GROW, NOAH!!!! There was no mention of concern for his central IV lines at this time since there continues to be no other blood cultures that have come up positive. He had some blood cultures drawn last night again for his fever, but these results won’t be back for another couple of days. However, the doctor did show some concern over the chest x-ray results. Apparently, Noah may have aspirated something at sometime or the ‘fluffy’ area noted around the heart may be something viral. They were not entirely sure at this time exactly what the results indicate, but they are thinking of doing a follow-up chest x-ray in a few days and possibly doing a respiratory viral battery test soon after to make sure it is not viral. He has not had any fevers throughout the day, so hopefully it is not viral, and nothing really serious at all. We will be praying and keeping our fingers crossed.
Physical Therapy was in today to work with Noah, and they brought a big red physioball for him to work on lying prone, on his belly. Noah has never tolerated lying on his stomach, for whatever reason, he does not like to be in that position. However, it is ideal to have him practice lying in that position so that may be one day he will become strong enough to lift his head, push up with his arms, and may be even try crawling one day. Who knows? The physioball is very forgiving and he can be very lightly bounced and rolled back and forth, and it seems to help calm him while he is lying prone on top of the ball, thus tolerating it for short periods of time. He tolerated it for approximately 45 minutes!! Go Noah!! We are responsible for working with him the most, so the ball is kept in the room and our space is already quite limited. However, it will be another task to add to our long list of exercises and care that we are already doing with Noah. Sometimes it is very tiring, especially when we have been up all night with Noah and don’t have a lot of energy to expend on doing all of his speech, occupational, and physical therapy exercises outside of the regular bedside care that we are doing. Hopefully he will begin to sleep better as he recovers from the mucocitis and chemotherapy, and his cells begin to engraft and grow.
Four other kids/families were discharged last week and three more this week. We are so very ready to join them, with high enough white counts to do a jailbreak! So GROW CELLS GROW Noah, so we can get out of here!! Thanks to everyone for the wonderful prayers, warm thoughts and kind words of encouragement. We are so appreciative of all of the great support that we have received during Noah’s fight for life. We sincerely ask that you will continue to remember him in your thoughts and prayers. God bless you all.

Tuesday, March 4, 2003 7:35 PM CST

Entry 37---March 4, 2003 Post Transplant Day 13

Today Noah’s WBC count was again 0.1, but at least it is not at 0. He has been given a lot of diuretics to help decrease some of the puffiness, most notable in his hands, feet and chin. However, with the increased water loss, he also loses a lot of potassium and his potassium blood levels have been low. He has required several potassium IV boluses throughout the day and has required frequent lab work to recheck the levels. Too little of potassium in the blood can SEVERELY affect the heart. For those aware, his last potassium level was 2.7 (approximately 4.0-5.5)!! Yikes!! His platelets were down to a 16 (norm approximately 150-400) and again required a unit of platelets this morning. He has had platelet infusions for two days in a row now. All of his other lab values are still relatively within acceptable ranges. He developed a rash all over today, and had his first fever of 101.2 (38.4 *C). He was able to cool himself down relatively quickly and did not require any meds to help relieve his fever. These are likely signs of engraftment and are not necessarily bad things. Hopefully we will see a rise in his WBCs soon. The doc came in and was pleased with what he saw. The occupational therapist, Lenore, came in with the Speech Therapist to assess the issue of Noah possibly aspirating his medications. We gave him one of his meds orally, and he swallowed it just fine and they both confirmed this. However, when given a little water, which has a lot thinner consistency than the medication, Noah had a slightly delayed swallow, but never choked on any of it. He will likely have a swallowing study in a couple of weeks after the mucocitis has subsided and he can come off some of the pain meds. We hesitate trying to test him now with too many variables, which might alter the results and lead to an inaccurate conclusion. For now, though, he will be allowed to take the medication by mouth if we prefer him to rather than using the NG tube. However, they are not going to pull the tube out yet even if it is not used very often just in case it is needed. We also were showed some exercises to help with oral stimulation, which will help him to maintain his sucking/swallowing reflexes necessary for bottle-feeding after he begins to recover. We have not heard anything new about a plan for his central IV lines and when Noah will need to go to surgery to have them replaced. As for now, that issue is still in limbo. Noah’s best buddy Heidi sat with him for a couple of hours this evening and they got along great. Heidi seems to enjoy cuddling him, and we know that Noah LOVES his cuddles time!! We are so grateful to have her and she is so very nice. She took some photos of Noah to hang up at her work place so that others can see her best buddy Noah. Overall, the day went fairly well, and we hope to see WBCs in the morning and hopefully it will be on the rise. His mucocitis is slightly better than it was over the last several days, and he does continue to spit up a lot of it. Hopefully he will be over this soon, because it seems to cause him some discomfort.

Monday, March 3, 2003 7:01 PM CST

Entry 36---March 3, 2003 Post Transplant Day 12

Noah’s white blood cell count today was 0.1! So at least it did not drop completely back to 0. Today has been another long day for us. Noah continues to have blood cultures drawn each morning with the other lab work, and so far no more have come up positive since the two that grew out 2 different types of bacteria on the 21st and the 24th. So we continue to question whether or not there was possible contamination that occurred because the types of bacteria that grew are common skin bacteria that would be otherwise harmless if Noah had an immune system. This combined with the fact that there have not been anymore positive blood cultures since then leads us to wonder if Noah truly has an infection in his blood at all. However, per protocol on the unit IV antibiotics were started immediately after the first blood cultures were drawn and these meds may actually be masking an infection that is still present. A doctor from the Infectious Disease department stopped in and suggested that both central IV lines should come out as soon as possible, and did admit that TAKING NOAH INTO SURGERY WOULD INDEED HINDER THE PROGRESSION OF HIS CELL ENGRAFTMENT!! She stated that if the lines were not pulled at this moment, then they absolutely must be pulled once his white count grows and stabilizes. We know that we have the last word on this issue, and we don’t want to place Noah in harms way by allowing an infected central line that leads into his heart to be kept inside his little body for any longer than is needed. However, since we have not seen any new positive blood cultures and the surgery under anesthesia would hinder his engraftment, we don’t want to go through with it right now. The doctors are continuing to discuss this problem and we hope that we will all soon be able to develop a plan with which we all agree on. In the meantime, Noah continues to not have any trouble with fevers and is still receiving his IV doses of antibiotics to treat any possible infection that he might have. He is supposed to have an Echocardiogram done soon to make sure that there is no infection affecting the function of his heart. We are not sure yet when this will be done, but most likely within the next day or so.
This is not the only issue we had to deal with today. Apparently the nurse on duty today felt that when she had given him some oral medication, he was not swallowing effectively and may have aspirated some of it. However, she did not bother to share her opinion with us. We were left completely unaware until the doctor came in to see Noah and mentioned that he thought Noah should have another nasogastric tube placed to prevent him from aspirating anything into his lungs due to ineffective swallowing. We were caught off guard and upset by this, because we were told the entire reason for taking the other NG tube out in the first place was that it could lead to an infection in the esophagus, called esophagitis, once the mucocitis (mouth sores) developed after chemotherapy. So our impression was that it was safer for him to not have one and we were left confused and concerned when we were told that he should get one placed today before his next scheduled dose of oral medication. However, the doc contested saying that having him aspirate and risk getting pneumonia would be worse than him getting an infection in his esophagus. What next? Needless to say we felt we had to concede, and Noah had an NG tube placed today for his oral meds to be given. It is frustrating having to take so many steps backwards before you can begin to go forwards, and we feel that all we have done is slide backwards. However, during our meetings with Dr. Kurtzberg we were informed that all kids seem to regress after chemotherapy/transplant for some time before they can move forward. So we aren’t completely unaware of this, but it isn’t easy to stand idly by and watch, because technically that is what we have been doing all along with his Krabbe’s progressing and his development regressing. We look to tomorrow for better news and pray that Noah comes out of this on top.

Monday, March 3, 2003 0:05 AM CST

Entry 35---March 2, 2003 Post Transplant Day 11

What a busy weekend it has been. Yesterday began with the news that one of Noah’s Central lines had somehow worked its way out the vein it was in and made its way out into a branch of that vein leading up the side of his neck. The surgeon mentioned that he had only read about his happening, but had never seen it until now. Are we surprised that Noah would be the one to show him this? Unbelievable! Initially this line was thought to be the one infected with ‘Staph’ bacteria, but actually it is the other central line with the double ports that has the ‘Staph’ infection in the line. At the same time, the double lumen central line had no blood return when attempting lab draws. A medication, TPA—a clot dissolver, was placed inside each line for two hours, but to no avail because blood could not be drawn from them. So the rumor was that Noah would need to go to surgery, possibly on March 3rd, to have the lines pulled and new ones placed. There is a risk for infection associated with the procedure, aside from the one he already has, and a risk for breathing complications from the anesthesia combined with his already compromised airway caused by the mucocitis. Meanwhile, he remained without a white blood cell count and we weren’t sure what we should consent to and what would be best for Noah at this point. However, we were given a day or so to think about it while the doctors and surgeons convened to discuss a logistical plan for Noah, as this was not necessarily an emergency at this time.

Noah slept better than expected during the night, but the nurse coming in & out a lot combined with the IV pumps beeping throughout the night, did not allow mom to get the best sleep. However, this did not matter after she learned that he has a white blood cell count of 0.3 again!!!! GROW CELLS GROW, Noah!!!!! She immediately jumped on the phone to call and wake up dad at the hotel with the GREAT news. When meeting today with the attending doctor today who is on call over the weekend, he mentioned that this rise in his WBCs is most likely NOT a fluke and that from now on we should not worry too much if the white cell count climbs and drops frequently, as this is quite normal. However, this is so very hard to do. Jeanne, our primary care nurse, was on today and fulfilled her promise to sit with Noah, allowing us to go out on the town for a while. So we took her up on this and went out to lunch to celebrate. Noah has done well today and we are anxious to see what his labs have to say in the morning. As for his central line issue, the doctors decided to wait for a while, as it may interfere and even hinder his engraftment progress, and we definitely don’t want to do that now. They said that this is something that will need to taken care of at some point in time within a few weeks, but not at this very moment. Another reason for postponing the surgery is because the engraftment process also has negative effects on the airway and can cause inflammation just as the mucocitis has done, which would present a problem when combined with the anesthesia required to do the procedure. So until then, GROW CELLS GROW!!! We are thankful for all of the prayers, as they ARE working for Noah, and despite the little mishaps, he is doing well. We are praying that the progression of the Krabbe’s will stop very soon and although it may be a long shot, we pray that he might ‘re-myelinate’ his nerves allowing him to have some of his lost abilities recovered. We realize that we won’t know for sure where he will be coming out of this for quite a very long time, but we continue to pray and hope.

Friday, February 28, 2003 8:31 PM CST

Entry 34---February 28, 2003 Post Transplant Day 9

Noah has a rash all over his body! This is a good sign. The doctor said today that it “looked like things were starting to happen.” We are hopeful that the labs will tell us the rest of the story we want to hear tomorrow. Noah’s color looked good today and he was really alert. It was nice to hold him and have him look at us without crying, he has done so well. Today our best buddy Heidi came to watch him so mom and dad could go to the store and get some items. We are very appreciative of her since the last time we got out together was when Kelly came to help. I posted a singe on our door that says, “Dancers wanted for, “Lord of the Cell Dance” performances daily.” The reason for the singe is because the nurses always joke that they will have to do a cell dance before they draw his labs to help the cells grow. I figured then the more dancers the better the odds. GROW CELLS GROW.

Thursday, February 27, 2003 11:07 PM CST

Entry 33---February 27, 2003 Post Transplant Day 8

The white cell count remains at 0, and we are desperately waiting to see it rise. Noah tested positive for 2 different types of bacteria in his blood, both are common skin bacteria that to most people remain of no threat. However, since Noah has no immune system, he is at risk for the infection to become life threatening if left untreated. He was started on antibiotics a few days ago, and continues to not have any fevers at this point, so we are hoping that it was caught early enough and it will be killed off very quickly. We were reassured that in no way will this infection hinder his stem cell engraphment, which is great to hear. He persists to vomit large amounts of mucous practically daily, but overall it seems to bring him some relief and he sounds a little less ‘rattlely’ for some time afterwards. Noah has no mouth sores, but we are certain that his poor little throat and the rest of his GI tract are extremely irritated and inflamed. He has demonstrated no real difficulty breathing yet, which is a relief. He does continue to sound very, very hoarse when he cries and has some nasal (nostril) flaring, but doesn’t exhibit any other signs of being in respiratory distress. The docs say that we are right on track, and that all of his lab values for his liver and kidney function look unremarkable. His blood count labs are on the lower side, but this is to be expected since his blood making capabilities were destroyed by the chemo, and his new stem cells are in the process of engraphing. Grow cells grow! We met our Best Buddy Heidi today and she is the sweetest person, and we are so very blessed to have her as our buddy. She has already offered to come and sit with Noah tomorrow after she gets off work! We almost don’t know what to do with ourselves!! However, mom was already given directions to one of the malls nearby from Jeanne, Noah’s primary care team nurse. So mom has a pretty good idea of what she can do with the time away from the hospital, but for some reason dad didn’t seem to excited. I wonder why? Regardless of what we do, it will be nice to get out and get some fresh air and see something other than these hospital walls for a change.

Wednesday, February 26, 2003 10:43 PM CST

Entry 32---February 26, 2003 Post Transplant Day 7

It’s been one week today since Noah’s transplant, and we are still waiting for his stem cells to make their appearance known. The influx in white blood cells yesterday was not necessarily a fluke, but his count today was back down to 0 again. So, we are not engraphing yet. It was so hard to go to sleep last night while waiting for the lab results, and once we were told, it was a great let down. Our disappointment was compiled with further frustration as we learned that another part of his blood culture lab came back positive, which means that he likely has an infection present in his blood. Since he has no immune system, this infection could turn into something really big and ugly. He has had no fevers as of yet, so we hope that the IV antibiotics that he was started on last night will begin to fight it off before it becomes severely threatening. Noah continues to sound like a barking seal when he cries because his airway is so very inflamed from the mucocitis. Many have compared this sound to a child with croup, which involves narrowing of the airway due to inflammation. However, the docs are not alarmed, and seem to be satisfied with his present status. We will continue trying not to get overworked about it. We shall wait and see what his labs have to show us in the morning. GROW CELLS, GROW!

Tuesday, February 25, 2003 10:29 PM CST

Entry 31---February 25, 2003 Post Transplant Day 6

We were abruptly awakened this morning at around 0400 by the nurse, after finally getting Noah to sleep and being up every hour throughout the night with him, and she delightfully informed us that Noah’s white cell count, which had been 0 all weekend, had increased slightly to 0.3! We were caught off guard and still half asleep before looking at one another in complete surprise and disbelief. During the day the attending doc stated that an early slight increase in white cells could indicate some that are floating around and just have not been disposed of yet by the body. However, on a very slim chance, the increase just might be the new stem cells engraphing!! We are told that it would be a new record. We will have to wait and see what tomorrow morning lab results will show us to be sure if there is a trend or not. His platelets are quite low and he will likely need another infusion in the morning, and his albumin is on its way down again as well. Noah is still vomiting mucous and sounds terrible, but not near as bad as yesterday. He has been a little more awake today than yesterday, and the flaccidity that he had yesterday has been replaced with some tone today. His throat is so irritated that when he cries, it sounds like he is hoarsely coughing. However, mom and dad are doing such great mouth care that he has not had any mouth sores develop yet, or maybe we are just getting a little lucky. We are trying to keep ourselves optimistic, but we are so exhausted trying to cope with disappointment and stress, that we have decided not to get overwrought about this heartening news. However, on a good note, the ‘wanted’ sign for a best buddy must have done the trick because it was replaced today with a sign reading ‘My Best Buddy is Heidi’, and included her picture. She is to call tomorrow to set up a time to meet us. She is a nurse at the VA hospital across the street from Duke, and has been a best buddy to other Krabbe families during their stay here for transplant. So she knows what behaviors that she can expect from Noah and is knowledgeable of the special precautions that must be taken to protect Noah and the other kids on the unit from contracting infection.
There are a couple of special donation accounts that have been established for Noah by some very thoughtful family members, as a means to show their support for us during this difficult time. We plan on using any donated funds to offset expenses while at Duke. Any funds left over when we return home to Hawaii will be donated to Hunter’s Hope. Again, we appreciate the mass of prayers for Noah and the encouraging guest book entries that so many of you have written to show your support. God bless you. GROW CELLS GROW!!!

Monday, February 24, 2003 7:20 PM CST

Entry 30---February 24, 2003 Post Transplant Day 5

Today Noah has been very lethargic and responding very little to any stimuli, of which he used to react to by crying and posturing. His color was very ashen and pale looking, rather than his normal rosy tone. He has not been coughing to clear his airway and has begun to really ‘rattle’ with his breathing, due to the increased mucous that is produced with the mucocitis. As well, the mucocitis causes inflammation and this can become dangerous, even life threatening, if the inflammation becomes so overwhelming and restricts his airflow. The doctors have been in several times today to check on him, which is unlike the previous days where they visited him only once each day for a short assessment, and this has heightened our awareness to the seriousness of their concerns. The docs stated that he will likely reach his worst at around 10-14 days post transplant, and that some babies have required to be transferred to the PICU, intubated and placed on a ventilator (life support) for a while due to the increased inflammation restricting the airway. This is something that we cannot even imagine at this point. Everything has been going so well up to now and we had a bad feeling that something was going to happen, because we could not get that lucky, especially given the year that we have had. We are not to that point yet, but it is very worrisome nonetheless. He continues to have swelling in hands/arms and feet/legs, and was started on Lasix, a diuretic, to help dry him out a bit. His PCA (pain med) was turned off for a few hours and his other sedation medications were placed on hold to see if his lethargy is related to having too much sedating medications on board, or if it is something more concerning. We hope that he will improve throughout the night and wake up a little more without completely reverting back to the behavior he had when he was first admitted. As we wait at his bedside to see how things progress, we continue to proclaim Noah’s battle cry, GROW CELLS GROW!!!, and we hope it will happen real soon!

Sunday, February 23, 2003 11:39 PM CST

Entry 29---February 23, 2003 Post Transplant Day 4

GROW CELLS GROW!!!! We were brusquely awakened this morning to Noah gagging, and it scared the heck out of us. Mom, being a PICU nurse and having slightly quicker reaction time than dad with these kinds of things, ran to the wall suction to help Noah clear his airway and keep him from choking and possibly aspirating on his mucous. He has had a lot more mucous in the last few days, and we are told this is normal and will likely persist for the first few weeks post chemo/transplant. Unfortunately, as a baby he doesn’t have the capability of sitting himself up or turning himself over to prevent himself from choking and aspirating, so he is at risk for aspiration pneumonia. He definitely doesn’t need this with his white cell count at a big zero. He continues to rattle when he breathes, but the mucous is in the back of his throat and not actually in his lungs. However, he doesn’t always cough to clear his airway, and this is when he gags. We keep him elevated all of the time and have him sleep on his side. Mom suctions him periodically, which does seem to help. He began running a slight fever this morning, but was quickly able to cool himself down without any interventions. He still is not eating, but remains on the TPN (HyperAl and Lipids) to give him the needed nutrients. We have been playing music for Noah, which seems to really help him to relax. He really enjoys his ‘bruddah IZ’ a lot, Israel Kamakawiwo’ole CDs that mom and dad brought for him from home in Hawaii. Since we were able to bring him home from the hospital, we would barbecue on our lanai and play one of the local Hawaiian radio stations and the music really soothed him. Mom and dad enjoy listening as well, and now Noah’s nurses enjoy the island sounds and we have seen some of them even attempt to hula. It is quite comical. Auntie Maggie’s mom and dad sent us another ‘local’ CD to add to our collection. However, Noah seems to react the most when mom and dad sing to him, and he gets a therapeutic baby massage with his bedtime lavender/chamomile lotion. That is something he definitely likes, and who wouldn’t? We are hoping that all of this will be beneficial in helping him to remain relaxed and for his cells to grow just a little faster than even the doctors are predicting. So far we have heard that the fastest they have seen the stem cells start working was approximately 2 weeks, but the stem cells were an ‘autogolous’ unit, meaning that they were harvested from the patient prior to his chemo and specially treated to take out the bad cells and then when time for the transplant arrived, they were reinfused into the patient. So the patient was his own donor, and the body did not have to readjust to something entirely new, as in Noah's case. The longest time noted for stem cells to start working as reported to us by the doctors, was almost 3 months! So we are praying that Noah will be able to miraculously beat their record for the earliest engraphment. We are still waiting for a best buddy, as it has already been 2 weeks that we have been inpatient. Maybe someone put the word out that it is a Krabbe kid that needs a buddy---oh no!! ;-) We were given some free gym passes and it would be a great opportunity to work off some heavy stress and give us a chance to get back into the shape we used to be. It is hard to believe that just a couple of years ago we did two marathons, yikes! Mom gets out of breath and exhausted just thinking about that! We placed a ‘Best Buddy Wanted’ sign on Noah’s door as a joke, because we know that the Family Support Rep is working on this issue for us, but until she comes through we are in lockdown, and going stir crazy. Hopefully we will find some relief soon. In the end it doesn’t matter, because even when we are out and away from Noah, he is ALL we can think and talk about, so we are never really away from him and that is the way it should be.

Saturday, February 22, 2003 11:50 PM CST

Entry 28---February 22, 2003 Post Transplant Day 3

GROW CELLS GROW!!!! Noah is holding strong, no fevers yet, and he still has a little bit of rash left over, but is doing relatively well. He has been in A LOT of pain and discomfort and has required a lot of Benadryl, Ativan, and Fentanyl to help try and keep him relaxed and calm. His doses have had to be increased a few times to try and meet his pain demand. His platelets, the blood component used for clotting, were very low today and he received a unit of platelets this morning. His white count is virtually 0 now, and so he is at his highest risk of getting sick since he no longer has an immune system to fight off germs. So, we are trying desperately to keep the room as clean as possible by wiping everything down with Clorox and Lysol each day. Anytime we pass someone and they happen to cough, we have this automatic reaction to hold our breath and move away from them as quickly as possible. As well, we have worn our hands raw from constant hand washing, but it is all worth it to prevent Noah from getting sick from something that we brought onto the unit and into his room. We know that the slightest little cold bug would be enough to possibly kill him, so we are extremely attentive about protecting him. The attending physician stated that Noah seems to be doing fine, considering all he has been through. So, GROW CELLS GROW!!!

Thursday, February 20, 2003 7:30 PM CST

Entry 27---February 20, 2003-Day 1 Post Transplant

GROW CELLS GROW!!!! Last night Noah was up a lot crying and had a lot of discomfort. Mom was with him in his room last night and tried to comfort him, but it wasn’t doing as much for him as the pain meds that they gave him. Dad arrived in the late morning and took over while mom went back to the hotel to try and sleep. While dad was with Noah, Dr. Martin came in to see him and said that Noah looks to be doing relatively well considering, without any mouth sores yet, and he started Noah on a Pain Controlled Analgesia pump with Fentanyl. It is a way that we can control Noah’s discomfort and pain without having to always call the nurse and wait for them to bring it and give it to him. This PCA has a button that we push whenever he seems to be in any discomfort and it is already hooked up to his IV fluids and is administered immediately. He can have some every 6 minutes as needed. This has been great and although he hasn’t needed it that much, it does allow us to keep on top of his pain fairly well. Noah has gotten a lot of cuddling from mom and dad lately, and maybe this will help his cells to grow faster. Thanks for all of the prayers for Noah, as his transplant is done and was successful thus far. It is still too early to know for sure, but getting this far is such an accomplishment.

Wednesday, February 19, 2003 9:35 PM CST

Entry 26, 19 Feb 03

GROW CELLS GROW!!!!!!, Today is Noah’s Life Day and he received his transplant at 1310 today. It was a small bag of stem cells given to him through his central line. The only side effects were a rise in his blood pressure and a drop in his heart rate. This was expected but caused Dad some stress. We now have to play the waiting game. We won’t know if the stem cells he was given today will be the ones producing the blood in his bone marrow until about day 25. We will be waiting on pins and needles until then. If the cells are female, then we know it is the donor cells, and they have engraphed. This means that the enzyme Noah is missing will be making its way to his brain, and stopping the damage caused by the disease. The biggest threat to Noah now is infection, because his immune system is totally gone. He has been doing well, we will continue to pray for positive results and appreciate all of your support and prayers.

Tuesday, February 18, 2003 11:36 PM CST

Entry 25---February 18, 2003

ONE DAY TO GO!!!! Here we are just less than 24 hours to go. He is finishing his last dose of ATG and doing well. He continues to have some lingering hives all over his body, but still has not had any fevers from the ATG. His transplant will likely be around 1100 NC time tomorrow. We will be taking a lot of pictures and posting them for everyone to see. A lot of the kids on the unit even have a picture of the bag of donor cells. Each child is given a certificate on transplant day, and we will be hanging Noah’s certificate on his door, as all the other children who have been transplanted have done. The most common phrase seen around the unit is ‘Grow Cells Grow’, so we added it to our collage of pictures and decorations. He impressed the Physical Therapist today by sitting up fairly straight in his ‘bassinette chair’ that we have made for him and sleeping for a few hours. He has been a lot more relaxed than usual and has even had his hands slightly open and the thumb on his right hand out, rather than clasped tightly in his palm. This is a huge accomplishment for him. It is likely due to all of the meds he is getting for relaxation rather than his own doing, but nonetheless it is great to see. We are really trying to be dedicated with working him on his PT and OT (occupational therapy) exercises as much as we can, and as much as he tolerates. We were told not to bother working with him too much during this time, because it is more important to focus on him getting his chemo and transplant, and that it probably won’t make much difference anyhow. We acknowledge this, but just the same, we feel that if we don’t work with him that we may end up losing some of his movements and skills that may have been able to be saved with frequent PT/OT. So we continue to have faith that it will help him in the long run. Besides, if nothing else, it will begin to get him acquainted with doing PT and allow us to develop a routine that will be helpful when he is outpatient. We pray that his transplant goes well, that his donor cells take hold and get to his brain to begin producing the much needed enzyme, and that in the meantime his Krabbe’s does not progress much if any to allow him a better quality of life.

Monday, February 17, 2003 10:12 PM CST

Entry 24---February 17, 2003

Today was Noah’s last day of chemotherapy!!!! We have made it this far, but there are so many more hurdles to overcome. Noah has done well. He still has some lingering hives from the ATG dose received yesterday, but he came through the first dose without truly getting a fever. His blood pressure was running on the low side throughout the day, most likely another side effect of the ATG. His IV fluids were increased a little to help expand his blood volume, thus elevating his blood pressure. As well, this helped to dilute his urine to prevent the remaining Cytoxan in his system from causing a severe bladder infection, as it has been known to do. Overall, he has done real well and again received a good report from Dr. Martin this afternoon. He said that Noah is right on track and is doing very well. He is irritable and seems to have some discomfort in addition to his typical Krabbe behaviors, however, we cannot imagine how irritable and uncomfortable we would be from undergoing chemotherapy. His nasogastric tube was finally removed late this evening after receiving his last dose of (oral) medication. He wasn’t too happy during all of this, but seemed to appreciate it being gone afterwards. From now on he will be required to take his oral meds by mouth rather than having the advantage of them being put down his tube and not having to taste them. This might just be a challenge for us, but he may prove us wrong. Tomorrow he will receive his final dose of the ATG immunosuppressant and it will technically be a day of “rest” from chemotherapy, as is written on his ‘transplant roadmap’. We shall see how much rest we all get, but just the same we will be relieved that his transplant is only a day away by that point! Please continue with all of your prayers as we near the big day and the long challenging road ahead of us that will be full of just ‘waiting’ to see what happens and when it will happen. This will be one of the toughest times for us, as we won’t know just where we will stand, and just how much damage the Krabbe’s has done before it is brought to a standstill. Of course we are hoping that little to no more damage is done from here on out, but it will all be revealed in time. We thank everyone who has been of such great support and has provided us with uplifting comfort.

Sunday, February 16, 2003 9:20 PM CST

Entry 23---February 16, 2003

Happy 5 month Birthday Noah!!! Today he received his third dose of Cytoxan, and began his ATG immunosuppressant therapy. He had a difficult afternoon and seemed to have a lot of discomfort. After giving him Ativan, Benadryl, Tylenol, and Zofran, he finally found some relief with Fentanyl! His liver is working overtime today! He only has one more day of the Cytoxan chemotherapy, and there are 2 days left for the ATG. The ATG comes from horse serum and is used to kill off the entire rest of his white blood cells, which are used to fight infections and anything ‘foreign’ to the body such as new donor stem cells. The ATG is given over 9 hours and can increase his irritability, as does pretty much everything else he has had up to this point! He developed a bad rash and his temperature increased in reaction to this drug, which is quite common. He has not developed any noticeable mucocitis (mouth sores) yet, but we continue to diligently perform his mouth care daily in hopes of preventing infection should they begin to appear. He still doesn’t care too much for this process, but has become a little more tolerant of it with each day. His eyes are beginning to get a little swollen, but his weight has remained relatively constant, so at this point there looks to be no actual fluid overload. He also began his steroid therapy today, which will last for the complete 100 days post-transplant. Over time he will likely develop the typical signs of steroid therapy, which include ‘chipmunk’ cheeks and excess hair on his back, upper lip or eyebrows. However, these adverse characteristics will fade and he will return to his normal adorable self over time. We are just 3 days away and very excited. We have so much riding on this.

Saturday, February 15, 2003 4:56 PM CST

Entry 22---February 15, 2003

A small miracle occurred last night, as Noah slept through the night for the first time this week. The increase of his medicine doses did the trick last night and we all had some much needed good sleep. Today was day 6 of chemo and he is still holding strong. His med-induced sleep and the chemo combined, caused him to have a lot of secretions and he sounded really congested this morning. However, after a rude awakening by the staff to check his vital signs and weight, his crying quickly cleared his airway relatively well. Pneumonia is a risk of having him snowed all of the time, so we only want him to be sedated enough at night to get some good sleep. So actually, his irritable crying is kind of good thing in a way. The nurse set up suction at his bedside just in case he continues to have increased secretions for safety. We definitely don’t need to be worrying about him getting pneumonia at a time when his immune system is being killed off. Kelly came by today and brought Noah a sweet little Valentine’s gift and a Hunter’s Hope tote bag, which made mom’s day. She stayed over 3 hours with Noah, allowing mom and dad to get out to eat a nice lunch and go do a little shopping. It was great to get out of ‘prison lockdown’ for a while, but all that we seemed to talk about was our sweet beautiful baby boy. We are relieved that we are almost to the day of transplant and are eager to see something positive transpire for a change. At times we feel like Job, and that we are always getting dealt the shoddy hand. In trying to remain positive and not feel sorry for ourselves, we continue to support each other and hope for the best; but there are so many uncertainties and we feel as if we are in the midst of a never-ending, vile storm. We won’t relent now, as we are too far into this journey to turn back, but things sometime seem so bleak. We know aren’t alone and after speaking with some of the other families here on the unit, our circumstances don’t seem as grave. We appreciate the enormous outpouring of prayers that we have received and ask that you will continue to remember Noah as he fights for life. He is holding strong, but the chemo is definitely taking its toll on his little body. Our little guy turns 5 months tomorrow and will be a day closer to transplant, and it cannot come soon enough.

Friday, February 14, 2003 8:22 PM CST

Entry 21---February 14, 2003

HAPPY VALENTINE’S DAY, HAPPY 6th BIRTHDAY HUNTER KELLY & HAPPY BIRTHDAY JIM KELLY!! Yesterday we finished up his last day of the Busulfan and started Cytoxan early this morning at 0400. He has not slept hardly at all, and so we are all very tired and frustrated. It was a rough night for us again last night, but with some changes made today in his medicine regimen, we are hoping that things will begin to improve. His major organ systems are holding up very well and continue to remain strong, and that is reassuring.

Wednesday, February 12, 2003 10:24 PM CST

Entry 20---February 12, 2003

Today was day three of chemotherapy for Noah, and it has been very rough, especially with his severe ‘chemorrhea’, as his dad has termed it. There is only one more day to go before starting his next phase of chemo. We were informed today by Dr. Martin, another attending that is covering the unit for the next two weeks, that the Bisulfan chemotherapy drug that he is getting right now is used to destroy the immature or newly made stem cells, that is why Noah’s labs show that he still has a relatively normal immune system. The next chemo drug is Cytotoxin and is used to kill off the mature immune cells already in his system and the rest of his bone marrow. He will get this drug for four days as well. The second chemo drug should not be quite as harsh on his GI system, or at least we hope not. Today was not very eventful, but he did relatively well. He did not go to bed until 0300 this morning and then woke mom up again at 0700. So once again, he did not allow her to sleep too long. Dad arrived soon after and she slept a few more hours. Auntie Amye showed up in the morning to help out and keep us company. Bless her heart, she stayed with us all day long and we are so grateful to have the company. Noah is holding strong with the chemo thus far and we pray that he continues to do so.

Wednesday, February 12, 2003 2:56 AM CST

Entry 19---February 11, 2003

Well, Noah awoke bright and early again, but did sleep about six hours through the night after finally getting some little help from his ‘candyman’ friends. Lately, it seems that meds are required all of the time now to keep him calm, relaxed, and to help him sleep, otherwise he is awake and crying inconsolably. Today they decided, with a little parental whining to help them make their decision, to place him on routinely scheduled doses of sedatives to help keep on top of his irritation. It worked wonders, for tonight he was awake and making good eye contact without constant crying. He fussed every so often, but at least he could be consoled. He had his second day of chemo, and it is doing a number on his bowels. Not to be too graphic, but he is having extreme diarrhea, that Greg calls ‘chemorrhea’, and this isn’t much fun for the whole family. He has been doing well with the chemo otherwise, only having one episode of vomiting. He has always been irritable so we’re not sure how much worse he can get, but we don’t really care to know. As well, his AM labs showed that his red blood cells (RBCs), his hemoglobin, and his hematocrit were low, so he did receive his first infusion of packed red blood cells this morning to help increase these values. His labs are drawn at least daily at 0200 hours, and then anytime thereafter as needed. He will have only 2 more days of this particular type of chemo before he begins the next one, bringing us ever so closely to day 0, transplant day!! Today we signed up for the ‘Best Buddies’ program where a specially trained volunteer is assigned to a patient/family to help them out by sitting with the patient and allowing the parents a break and various other things to help out the assigned family. We hope that this will allow Greg and I to actually get out for a little while. This would be of GREAT help to us, as we are growing very tired. Mom’s long time friend since preschool came all the way from Wisconsin just to meet Noah and to see his mom and dad, whom she hasn’t seen since their wedding over 6 years ago. She is also a Peds RN like Noah’s mom. It’s been great visiting with her today. We are delighted that despite the tragic reason for having to be here, that we are able to visit and see family and friends that we have not seen in a long time, especially now that we have Noah. He is such a very special baby boy, as all parents profess about their children, but he has such a sweet spirit and seems to have touched so many lives in so many ways. We would like to send out a reminder that on February 14th, it is not only St. Valentine’s Day, but also Hunter Kelly’s 6th birthday, which brings about a HUGE celebration. We were given a special luminary candle from Kelly for this very occasion and just wanted to pass on the news about this great event. For those unaware, Hunter Kelly, son of NFL’s Jim Kelly, has Krabbe’s and has not had a transplant. His road has not been easy, as he has been sick several times, but from the pictures I have seen of him from this past year, he looks great!!! Well, we will continue to keep everyone posted about his progress this week with his chemotherapy.

Monday, February 10, 2003 7:46 PM CST

Entry 18---February 10, 2003

Today Noah started his chemotherapy at 0400 hours. The first chemotherapy drug he is getting is Busulfran through his nasogastric feeding tube. A common and serious side effect of this drug is seizures, so Noah is also taking Dilantin to prevent him from having any seizure activity. He has still been very irritable and continues to require sedation frequently to attempt to keep him calm. He did not sleep well last night, and was several hours crying and inconsolable. So he has slept a lot today to make up for being awake all night long. Mom was not very bright and cheery this morning, as she was the first to stay overnight with Noah in his room. A dinner was catered last night by a local organization that donates a meal monthly to family members of the patients, and we were able to get away and join some of the other parents to eat a hot meal. We seem to be the only Krabbe family on the floor, but all of the patients on the unit are undergoing bone marrow/stem cell transplants for their particular disease, so we do have that in common with them. They are very nice and friendly. All throughout the day, Noah has been ‘bothered’ by occupational therapy (OT), physical therapy (PT), doctors, nurses, child life & family activities specialists, etc. and wasn’t too thrilled with all of the attention. He has done relatively well today with his first day of chemotherapy, but we are only in the very beginning stages. We also have bothered him quite often today to do his regular mouth care. Almost all patients undergoing chemotherapy develop mouth sores, also known as mucocitis, and so they are required to perform frequent mouth rinsing with special solutions to help prevent the sores from becoming infected. Noah doesn’t like it too much, but he doesn’t like too much about this whole ordeal and we don’t blame him. We will be responsible for a lot of his care, such as doing his physical & occupational therapies with him regularly and frequent mouth care. So we will be keeping really busy. Our plan at this point is to start aggressively working with him on his OT and PT to keep him mobile as much as possible. He doesn’t move or bend his legs much anymore and he typically keeps his arms, head, and back extended almost all of the time now. Jenn, the physical therapist taught us some techniques to properly help him to become more flexed and relaxed. He isn’t getting into it yet, but with time and some work, he may become more comfortable with it, which will make it much easier on us. As of now, he attempts to fight against us and keep himself in an extended position, and he is quite strong. We spent some time today decorating his door with pictures, and used construction paper to cut out his name and ‘aloha’ and place them on the door with a big red hibiscus flower to share a little Hawaii with everyone on the floor. Dad spent a few hours taking advantage of the Sony Playstation II that is in the room. Those of you who know Greg know that he will definitely make use of it. Mom spends some of her time watching TV, reading magazines and working on her quilting/cross-stitching projects. However, Noah doesn’t let mom and dad get away with playing around too much. Well, we shall have to wait and see what tomorrow holds for us. Hopefully it will be a much quieter night.

Sunday, February 9, 2003 8:24 PM CST

Entry 17---February 9, 2003

Aloha. We arrived today on the Pediatric Bone Marrow Transplant Unit (PBMTU) at Duke and found our way into room #5205 where Noah will remain throughout the duration of his stay. He had his admission labs drawn and his IV fluids started. He had an EKG done soon after arrival. He was too thrilled about all of the poking and prodding that went on for the first few hours. We have added his room # and the phone# to his room at the bottom under the hospital information, since we will now be spending most of the hours each day in his room, but if we aren’t there, we can probably be reached via cell phone or in the hotel room. We are relieved to be here and yet, are a little apprehensive about his chemotherapy, which will begin in the morning around 0400 AM. We have eagerly and impatiently waiting for today for the last few weeks and it almost seems like a dream now that it has arrived. Dr. Kurtzberg popped in to make a quick visit and to answer any more questions that we may have. We expressed our enthusiasm to get things started. Noah’s primary nurse, Jeanne, who is very nice and extremely helpful, greeted us. Everyone has been extremely supportive and friendly. We are blessed to be here at Duke, where they care so much about saving little Noah’s life from this horrendous disease. God bless them.

Saturday, February 8, 2003 1:58 PM CST

Entry 16---February 8, 2003

Well, we are down to one day before admission, and it can’t come soon enough. Noah has been really irritable and difficult these last couple days, and we are growing very weary of being stuck in this hotel room, just waiting for the hours and days to pass by. At times, we wonder how we made it this long without going nuts. It has been a challenge trying to explain what we have been going through for the last 4 months, but for those of you with Krabbe kids, it is all too familiar. Nonetheless, we appreciate the wonderful support that EVERYONE, across the nation, has shown us. In the end, when all is said and done, we will never be able to show enough gratitude for the enormous outpouring of love and kindness shown to us, but we want everyone to know that we are very thankful and extremely touched by it all. Mahalo and God bless.

Friday, February 7, 2003 12:39 AM CST

Entry 15---February 7, 2003

Today Noah went to clinic to have some final labs drawn before being admitted on Sunday afternoon to start chemotherapy this next week. The CMV lab was checked on Wednesday and has come back as being negative, and his white blood cell and hemoglobin were low, most likely due to his CMV medication. This doesn’t matter since the chemotherapy will be killing off all of his bone marrow anyhow. Kelly has offered to watch him for a few hours so that we can get away for a short while before Noah goes into the hospital. We are considering taking her up on this offer since we have been locked in this room or the clinic for the last three weeks with no break in site. We would hesitate under normal circumstances to leave him with anyone due to his special care needs but Kelly is well acquainted with the challenges of a Krabbe kid and we trust her ability to deal with and care for Noah’s unique needs. She is such a saint and we appreciate all that she has done for us.

Friday, February 7, 2003 12:23 AM CST

Entry 15---February 7, 2003

Today Noah went to clinic to have some final labs drawn before being admitted on Sunday afternoon to start chemotherapy this next week. The CMV lab was checked on Wednesday and has come back as being negative, and his white blood cell and hemoglobin were low, most likely due to his CMV medication. This doesn’t matter since the chemotherapy will be killing off all of his bone marrow anyhow. Kelly has offered to watch him for a few hours so that we can get away for a short while before Noah goes into the hospital, and we are considering taking her up on it. We would hesitate leaving him with anyone, just because he is not the typical baby and can sometimes be absolutely impossible to deal with when he gets into his irritable mode and we have a lot of difficulty getting him out of it ourselves. We can only imagine how hard it would be to deal with, as someone not acquainted with his ‘normal’ behaviors. However, Kelly had a baby with Krabbe’s disease and is very aware of what this disease is all about. She is such a saint and we appreciate all that she has done for us.

Wednesday, February 5, 2003 8:10 PM CST

Entry 14---February 5, 2003

Aloha. We bring good news!! We met with Dr. Kurtzberg (a.k.a. blessing of heaven) today and she shared with us that she has located Noah’s donor stem cells. They are coming this week from UCLA and the match is a 5 out of 6, NOT a Krabbe carrier, and the enzyme level is a 4.5!!! As was described to us, ‘normal’ levels of the enzyme range from 1-6, depending on the individual---some of us have more enzyme than others for whatever reason. BUT, although there is no actual proof, the likely assumption would be that since he has NONE of this enzyme, the more that he gets, then maybe the better the chance of stopping the disease progression and maybe, just maybe even slightly reverse some of the effects that he has already exhibited. So we are extremely pleased to hear all of this! We have a long hard road ahead, with a lot of other difficult and scary hurdles to overcome, BUT we are definitely hopeful. We forgot to mention in the last journal that we had the privilege of meeting Gina Rugari, Gina’s mom Anne, and Micki Gartzke who is the Education and Awareness Director for Hunter’s Hope. They are here for Gina’s 3-year check-up with Dr. Kurtzberg. Anne has been working on collecting worldwide demographic research of Krabbe’s disease for Hunter’s Hope. Unfortunately, Krabbe’s is frequently diagnosed too late in the progression of the disease, at time of autopsy, or never diagnosed at all. As well, when a child passes of complications, such as pneumonia, then rather than recording the death in association with Krabbe’s, it is only diagnosed as pneumonia. Thus, this means that there is an inaccuracy of the reporting of the disease and alter statistics of the occurrence of Krabbe’s. It is very likely that this disease, although quite rare, may not be as rare as we think it is. The solution for this, EDUCATION! So as we have mentioned previously, if there is anything that we could ask anyone to help us out with, it would be prayers for Noah and educating yourselves and everyone you know about this disease, including your health care providers. It is the best method to help out others who may and will have to go through the heartache of learning that their little child has a fatal disease. We know very well how devastating this is and do not wish it on anyone. Anyhow, we enjoyed visiting with Noah’s grandpa and grandma Fetterhoff for a few days. They left today to return to Missouri, in hopes of getting home before the bad weather moving this way sets in. We were pleased that they had the opportunity to meet and get to know Noah for the first time. We had a great time.

Monday, February 3, 2003 5:57 PM CST

Entry13---February 3, 2003

Today Noah’s Grandpa and Grandma Fetterhoff drove in from Columbia, Missouri to visit. They had not met Noah yet, so they felt it a perfect time to come and get to know him before he undergoes his chemotherapy and transplant this coming week. Greg and Deb have not seen them since they left Missouri for Hawaii 5 years ago. They will be here for a few days before having to head back home due to work. Noah has done really well the last 2 days, and not quite as irritable as he was throughout last week. We are eager to start his chemo and even more so for his transplant. We will meet with Dr. Kurtzberg on Wednesday to learn of the donor stem cells that he will receive and to sign consents for his procedures. We look forward to this opportunity to save Noah’s life and are praying that all goes well.

Saturday, February 1, 2003 6:49 PM CST

We wanted to share with you a special sermon prepared and given last Sunday by our pastor at Our Savior Lutheran Church in Aiea, HI. We were extremely touched by it and felt a need to share it with all of you.

A mom and dad are sitting in the living room watching their children play. The boy is busy with a Nintendo and the little girl is pretending to be a ballerina. As the children play, the parents daydream of their children's
future. The husband sees his son as a successful computer programmer. The wife pictures her daughter as a ballerina. Suddenly the godlike announcer cuts in and says, "how can you guarantee your children's future? You
need to plan today!" And then of course they show a particular insurance company whose product will guarantee this family will never have to worry about anything again - if they will just call today. It's important to think
about the future. It's important to make plans.

Two weeks ago one of our families found out the hard way the cost of waiting. Deb and Greg had been taking their baby to the doctor because of his irritability and occasional muscle spasms. The doctors assured them time
after time it was just colic. Finally, a doctor ran a few more tests and little Noah tested positive for Krabbe's disease. A neurological disorder that is caused by the lack of a particular enzyme in the body. It's exceptionally rare and fatal.

When I arrived at the hospital the doctor was explaining some of the terrible things they had to prepare themselves for as they watched their little four month old child die before their eyes. And to make a terrible thing even worse, the doctor said Noah could live two years or longer
during which time he would slowly lose his eyesight, hearing and ability to move his arms and legs. We all sat there in silence, shocked and overwhelmed as little Noah
slept off the anesthesia from the MRI. We prayed. We read scripture. We cried. The parents asked me to make a few phone calls because there were some things that they hadn't gotten around to because they assumed there would be
plenty of time. One of them was a life insurance plan. They knew they needed one - but you assume you're in no hurry because after all he has his whole life ahead of him. I made the phone call and while sympathetic and
understanding - no company in their right mind would give a policy to a child who has been diagnosed with a fatal disease - even if the policy was just so the family could afford the funeral costs. The next day I told the family what the company had said - they understood. I also promised them we'd find a way to do what needed to be done when the time came. Nobody should have to worry about such things and certainly not when there were more important things to take care of.

Two months ago, little Noah was held over that font and we poured water on his brow and spoke the words, "I baptize you in the name of the Father, Son and Holy Spirit." You were a part of that ceremony. God told Noah, "follow
me." And your part in the baptism was to let that tiny child know he would not be alone in his journey. I want to remind us all of something - God never promised anything to Noah except eternal life in Jesus' name.

I want to take you back into the Gospel lesson for a minute. Do you see the beginning words, "After John was put in prison, Jesus went into Galilee, proclaiming the good news of God. "The time has come," he said. "The
kingdom of God is near. Repent and believe the good news!"
Do you see the irony? Do you see the complication? "After John was put in prison - Jesus said "the Kingdom of God is near - Repent and believe the good news." It doesn't sound like good news to John - and we know it gets worse because old King Herod is going to behead him. Where's the good
news in that?

I ask the same question of little Noah. Where's the good news? To find an answer, we must concentrate on the words, "The kingdom has come!" It may not always look like it - but God's kingdom was ushered in when the ladies went to the tomb on that first Easter Sunday and found nothing
there. Before that moment - the only thing the people of God had was a promise. And promises are great...but sometimes they can seem pretty empty as we wait and wait and wait for them to be fulfilled. Those ladies along with
Peter, James, John and the rest of the gang were done waiting. Somewhere between Good Friday and Easter Sunday God fulfilled His promise and anyone and everyone who called on His Name - anyone and everyone who believed the
promise - would be saved. It's right there in the Bible, "whoever calls on the name of the Lord shall be saved." The next question is, saved from what?
You and me - we have a long list of things we expect to be saved from. If we belong to God - if we believe - we expect to be treated differently than the rest of the world. After all - isn't God on our side now that we're on
His?

When the same John the Baptizer we're talking about earlier was sitting in prison - he sent his disciples to Jesus to ask a simple question, "are you the Messiah or should we look for someone else?" And Jesus answered, "Go back and report to John what you have seen and heard: The blind receive sight, the lame walk, those who have leprosy are cured, the deaf hear, the dead are raised, and the good news is preached to the poor." As great an answer as they may have been - it didn't do John any good. He was still
in prison. Promise fulfilled or promise broken?

When God says, "follow Me..." He doesn't outline everything such a following entails. He doesn't tell us where our path will lead or who we will run into along the way or what trials and temptations we might face or
when the earthly part of the journey will end. He simply says "follow Me..." and expects us to follow. There is a problem. We often assume this following is like finding the
bullseye in a target. The center is always in the middle. And if we're a little low or to the left we can just keep adjusting until we get it right. And once we find it - we can just stay there. But that isn't the kingdom. In God's kingdom, the kingdom we live in here and now, the center keeps moving because the center is wherever Jesus is.
And Jesus is where we need to be. This may help explain why you may feel a little uncomfortable about your faith sometimes. It's those moments where one minute we felt so close to God and then the next it's like we're miles
and miles away and we have to start all over again trying to find the bullseye.

In our journey as Christians, we are all given the opportunity to move closer to the center of the kingdom, to where Jesus is. But constantly moving toward the center of the kingdom often calls for a radical change in direction. And those changes in direction can be painful when we aren't
prepared. Please note the radical changes in direction are necessary because we lose focus and get complacent. The only good news is, the longer you are a Christian - and the more you are immersed in God's Word and Spirit - the
better you are at anticipating which way the bullseye is going to go. And as you learn to anticipate, you also learn to watch for the "why?" The bullseye doesn't move indiscriminately. It isn't random. God has a purpose
why it moves and where it moves and the purpose is directly related to you and your life and where you need to be.

When a 16 year old gets his driver's license and goes with his parents to the car dealership to pick out his first car - he might be over looking at the new bright red Ford Mustang 5.0 convertible with leather interior and racing stripes - but his parents are on the used lot looking at a 87 Nissan Sentra with faded paint and a hole in the dash where the stereo use to be. The son might be yelling at his parents to come and see what he wants - but eventually the son will have to come to where his parents are and see what they decided he needs.

So often I stand looking at something and I'm calling to God, waving like crazy for Him to come and see what I want. And when He doesn't move, when He stands there motioning for me to join Him - when I see what He has
in mind or where He wants me to go - I start yelling, "but God - You promised!" And that's when God says, "yes I did - but let's look at what I promised - let's talk about what I really said." I don't like to hear those words because I know where they are going to lead. God promised me His love. He promised me forgiveness of sins. He promised me I wouldn't be alone no matter where I went in this world
and He promised me eternal life when this life is over. That's it. He didn't make any promises about the when, where, how and why except that He would be with me every step of the way. And if I needed a reminder - did you hear the lesson from Jonah. Boy does that sound great. "Then the word of the LORD came to Jonah a second time: 'Go to the great city of Nineveh and proclaim to it the message I give you.' Jonah obeyed the word of the LORD. The Ninevites believed God. They declared a fast, and all of them, from the greatest to the least, put on sackcloth and when God saw what they did and how they turned from their
evil ways, he had compassion and did not bring upon them the destruction he had threatened."

That's a great passage because we skipped all the stuff about Jonah running away and getting swallowed by a giant fish and getting belched up on shore covered with fish slime and seaweed. We skipped the part about Jonah not
wanting the Ninevites to be saved because he didn't like them and how at the end of the story Jonah is still mad at God because He saved them. Do you see how it worked? God wanted the Ninevites to be saved. He wanted Jonah to be the messenger. And Jonah ran all over the place trying to
avoid God before He finally gave in and joined God in the bullseye. And if Jonah prayed the prayer, "not my will but thine be done" he did it with gritted teeth and clinched fists because it's not what he wanted. There's a lesson
in there for us if we're paying attention because more often than not when we join God in the center it's not because we want to be there but because we finally gave up trying to get Him to come our direction. I always
wondered if some time later when he was sitting in the old prophet's home rocking away if he ever got it - if he ever figured out what a great thing God did through him.

We can never forget that living in the grace and will of God involves a cost. There is no cost for the forgiveness and love of God because His Son already paid that price on the cross - but to live the Christian life in the midst of world that cares for little for God and His will - the price can be horribly high. The first disciples of Jesus recognized that cost when they left everything they had to follow Jesus. I think too often we assume the things we must give up, the things we must be ready to live without are exactly that - things. But that would be too easy. I love the things I have in this world - but just as St. Paul
says, they are rubbish compared to my faith.

How often I have sat with grieving families who would gladly and willingly give up all they own if they could get God to bring their loved one back to life or restore the health of their little baby. "God, I'll give you
whatever you want if you will just do this for me..."
And the bargaining is well intentioned. It's that rare moment when we truly understand the value of our things and the importance of those we love. At almost any other time we hold tightly to the things of this world, we're so
proud of what we have and we would fight to protect it and keep it - but then in the twinkling of an eye the things are suddenly not important at all and we'd trade it all and so much more if we could only get what we really wanted.

There are millions of stories of people out there who when tragedy struck gave up their faith. When Jesus said, "follow me," they followed. It's easy to be a Christian when everything is going the way it's supposed
to, which is the way you think they should go. But I suppose we have to point out that such a faith is really no faith at all. When everything is going perfectly - the only thing you need to believe in is yourself. But when bad things happen. When it comes down to a choice between
faith in what God really promised and the promises we think or wish He made, invariably we resort to standing in front of the Mustang 5.0 convertible and gesturing for God to come over to where we're at. And we'll stand there
stubbornly - waving, jumping up and down, yelling to get His attention. And when it's obvious it's not going to work - that if we want to be with Him we're going to have to go where He is - it's not uncommon for someone to say, "Hey God, I wouldn't have followed you if I'd known where we were going to wind up."

And there is the mistake. The mistake is in thinking that because you followed God - because you belonged to Him and believed in Him when these terrible things happened - it's His fault. Your faith and trust brought these terrible things into your life. If God had never said, "follow
me," if you'd never heard of Him and never followed Him - your life would have been perfect and everything would have turned out just the way you wanted. But that isn't the way it is or would be.

Your journey, your path, your life as a Christian is exactly what it was going to be. The difference - the huge difference is you don't have to go through any of it alone.
It doesn't matter whether you are a Christian or not - people in your life are going to get sick and die and someday you're going to get sick and die. Airplanes are going to crash into peaceful neighborhoods and crazy
people are going to start wars and companies are going to go bankrupt and earthquakes are going to shake cities and little babies are going to be diagnosed with terrible diseases. And through it all, in it all, over it all - God makes some very simple promises: He loves you, He has forgiven your sins, when this life is over He wants you to live with Him in heaven AND you will never, ever be alone -
no matter where you go and no matter what you're going through. Those are the promises He made to you and those are the promises He intends to keep.

In far fewer words and less time, I shared these words and promises with Deb and Greg and little Noah up in the ICU at Tripler. Having laid the foundation we then prayed. If Noah's life was only to be a few years and if those years were to be filled with pain - at least he would not be
alone for one minute of his life - because God made a promise. I went back to the office and began contacting a few folks so we could set up a support group for the family. Meals, babysitting, finances. I spoke to a few nurses to get some more information and downloaded a few
articles off the Internet. If you've ever watched a football game between the Buffalo Bills and whoever your favorite team is - you may have seen the commercial with Jim Kelly and his son, Hunter. Hunter has Krabbe's disease - diagnosed just like little Noah at 4 months of life. Jim Kelly started a foundation - it's called
Hunter's Hope and it's goal is to do whatever it can for those who are diagnosed with Krabbe's. I sent them an e-mail and to my surprise Jim Kelly's mother in law responded
telling us the family would be in her prayers. I printed the sheet and thought it might bring a little comfort to the family as they sat in the ICU.

It was then that something miraculous happened. You see our e-mail wasn't confined to "delete folder" and quickly forgotten. It was forwarded to the head of the foundation who picked up the phone and called the Army Colonel who was in charge of treating Noah. In short, she said she wanted Noah on a plane within 72 hours so he could begin an experimental treatment at Duke University in Durham, North Carolina. We all know how quickly the military moves and despite the plea that every day wasted meant less of a chance for Noah - we assumed it would be months before orders could be cut.

Less than 48 hours after the phone call, the Air Force diverted a C-17 cargo plane to Hickam AFB where it was to pick up it's entire cargo of one mother, one father and one very tiny little baby. Greg sent me pictures of the
cavernous airplane and there in the middle little Noah on a blanket with a few toys surrounding him. 9 hours after wheels up the plane landed in Durham and a specialized crew was waiting to take Noah to the hospital.

They poked and prodded and took blood tests and more MRI's and Cat Scans and finally came to the conclusion that he's a candidate for bone marrow transplant which might - just might - keep this horrible disease at bay. And the doctor said while his life might not be exactly what they
imagined - if all goes well he will at least have a life. Noah begins chemotherapy on Monday and is waiting for bone marrow match for the transplant. The Army called all of these happenstances - we believe something different.

You see we know what promises God made to Noah - but it appears He just might have a surprise or two in store and maybe, just maybe we won't need that life insurance policy yet - all we can do is wait and see - in the name of the
Father, Son and Holy Spirit. Amen.

Saturday, February 01, 2003 at 12:18 AM (CST)

Entry 11---January 31, 2003---CORRECTION

Not much of anything exciting happened today except Noah had to get his nasogastric tube changed and replaced with a new one. He didn’t care too much for that, and we don’t blame him. Dr. Kurtzberg found 4 possible donor cells that match Noah’s bone marrow type, and has sent them off to be tested to determine the percentage of the enzyme that is missing in Noah’s blood. She will likely choose the one with the most enzyme level, to give Noah the best chance. We should know next week when she returns from out of town. Until then we won’t be doing much of anything. We plan on sharing a special message with you this weekend that was given to us by someone held dear to us. Please be watching for it tomorrow. God bless.

Friday, January 31, 2003 at 08:29 PM (CST)

Entry 11---January 31, 2003

Not much of anything exciting happened today except Noah had to get his nasogastric tube changed and replaced with a new one. He didn’t care too much for that, and we don’t blame him. Dr. Kurtzberg found 4 possible donor cells that match Noah’s bone marrow type, and has sent them off to be tested to determine the percentage of the enzyme that is missing in Noah’s blood. She will likely choose the one with the most enzyme level, to give Noah the best chance. We should know next week when she returns from out of town. Until then we won’t be doing much of anything, and most likely will not be writing a journal entry this weekend. God bless.

Thursday, January 30, 2003 at 04:45 PM (CST)

Entry 10---January 30, 2003

Noah had his last two major tests today, his audiology and opthamology tests, before he is admitted to start his chemotherapy. As far as the doctors performing his tests could tell, his hearing and eyesight are fine. His results don’t appear exactly normal, but because of the deterioration of the myelin in various areas of the brain, they were not expected to. The tests are not looking for the neurological function as much as just his ability to actually see and hear, and he can at this point. He does exhibit signs of slow tracking of objects, which is due to the Krabbe’s. We are relieved to learn that he can hear and see at this point. Getting Noah’s test results has created a lot of anxiety, as we don’t often receive good news. His irritability has really increased throughout this week and has been another contributor to our anxiety. We cannot wait to start his chemo and get this process going. We are looking at about 10 more days until he is admitted. His stem cells should be ready for him by this weekend and waiting for him until day of transplant. We will be meeting with Dr. Kurtzberg next week to sign consent forms that were not signed this last Monday due to his CMV and postponement of his chemo/transplant. Until then we will be hibernating indoors to stay warm and to prevent Noah from getting sick. Noah is holding strong and we are trying to follow his example and be strong for him.

Wednesday, January 29, 2003 at 05:08 PM (CST)

Entry 9

This morning we went to the pediatric bone marrow clinic as we have done pretty much daily, for the nursing staff to change Noah’s chest dressing and give him his IV medicine for his CMV. He did very well and all looks good. We are going to be taught how to care for his central line sites ourselves, including dressing changes. As well, we will be taught how to administer his IV med ‘at home’, so that we won’t have to go to the clinic twice a day to have it done. This will be old hat for mom, but will be a new learning experience for dad, altogether a unique experience since it is on our own child. Tomorrow he will have his audiology and opthamology appointments, which were rescheduled due to bad weather last week, causing cancellations. We are all trying to be strong, at times relying on all of the very encouraging and uplifting guestbook entries and emails that we have received. Thank you everyone for all of the much appreciated encouragement. Although we recognize the necessity of the testing that Noah has undergone over the last couple of weeks, when the results are discussed with us, it has been extremely alarming, discouraging and has caused us a lot of anxiety. We haven’t shared all that we have been told, because the doctors are speculating what Noah’s outcome and prognosis will be, however, it won’t be known for sure until we get there. And as we are all aware, if it be God’s will, he could surpass these speculations and surprise us all, it is just too early to say and so we would rather continue to allow us all to stay optimistic and have faith that Noah will be a miracle. So please continue your prayers and we will do our best to continue to keep you up to date on Noah’s progress. God bless you all.

Tuesday, January 28, 2003 at 05:51 PM (CST)

Entry 8, Jan. 28th, 2003

Today got off to a very rough start for us all. Greg woke up in the middle of the night, extremely sick. So, unfortunately, he was unable to go with Noah today for his outpatient surgery. So mom carried Noah over early this morning to the pediatric surgical clinic to check in. Noah was wisked away by the anesthesiologist and was in surgery a total of just under 2 hours. He had a double lumen left chest Hickman and a single lumen right chest Broviac catheters that will be used for the duration of his care here at Duke. Basically, in layman terms, they are 2 larger size ‘IV’s placed directly into the main veins in the chest that lead into the right atrium of the heart. These type of ‘IV’s can be used for months and will prevent Noah from having to receive needle sticks with the daily laboratory blood draws that he will need to monitor his blood counts and other various labs. However, he does have to endure the temporary pain and discomfort from this procedure. He also had a small skin biopsy done for research purposes and another spinal tap done to recheck the protein in his spinal fluid. It was previously 247 (normal of 10-40) upon his admission to the hospital in HI. Today’s protein value was 280, so it has not increased that much yet, which is a relief. Mom met him the recovery room, where he was in some pain and given some strong pain meds to comfort him. We were discharged from there and went back to the clinic where he received his first dose of the medicine he is receiving for the positive CMV result as mentioned in yesterday’s entry. Finally, after several hours at the hospital, we finally made it home 7 hours later to find dad still feeling very bad. Tomorrow he will return to the clinic early to have his Central line dressing changed and another dose of his CMV medicine. He did very well today and was very brave.

Monday, January 27, 2003 at 08:23 PM (CST)

Entry 7, 27 Jan 03

Today was a difficult day for us. Noah had been unusually agitated last night and Debbie and I really were only able to sleep from around 0300 to 0430. Needless to say the wait at the clinics today seemed to drag on forever. Noah’s echocardiogram results were normal, so we were thankful for that; chemo is hard on the heart and he will need all the advantage he can get. We were told that morning that Dr. Kurtzberg wanted more blood tests this morning but we didn’t know why. Unfortunately we found out at the end of the day when we meet with her that Noah’s blood work from earlier in the week tested positive for CMV, a virus. It requires treatment before the transplant process so it doesn’t kill him during the suppression of his immune system. This will postpone the start of chemotherapy by one week from 2 Feb to 9 Feb, and the transplant from 12 Feb to 19 Feb. This was hard for us to hear because we feel like we are constantly fighting the clock, and one week is a lot of lost time for Noah. We can only hope and pray that this is the only set back we will encounter.

Sunday, January 26, 2003 at 09:15 PM (CST)

Entry 6, 26 Jan 03

Noah went into the clinic Saturday and had to have an NG tube put in because he has not been eating. After placement of the tube, he has gained some weight from 5.9 kg on Saturday to 6.1 kg today. We can’t figure out why he is not eating, this all seemed to happen after two consecutive days of sedation for his MRI and EEG/evoked potential tests. He is still able to take the bottle so we don’t think it’s the disease. He acts like it causes him some discomfort to eat with the bottle. He is able to swallow and has not been choking on the formula, so it is very puzzling for us. It could be that all of the medicines he is on are causing him some stomach upset. Tomorrow he gets his echocardiogram, pre-op consult, and then meets with Dr. Kurtzberg for the look at all of his test results and to make the final decision to go ahead. We will then sign the consent forms. It will be a busy day for all of us tomorrow. I will update the journal with the results of our discussion with Dr. Kurtzberg tomorrow evening.

Friday, January 24, 2003 at 06:33 PM (CST)

Entry 5
Today Noah had his nerve conduction study (see photos). He was so tired because he had been sedated for the last two days that he slept through it. We also took him to see his Nurse Practitioner; she explained the process of chemotherapy to us and gave us the consent forms to go over this weekend. There are some risks involved, but we understand the consequences if we do nothing, so we are going to move forward. His MRI came back and showed no damage to his brain stem and that the disease has not made any noticeable progress in the last week. Also his viral test was clear, so there will be no delays. This was a big relief for us. His bone marrow type has been put in to the national registry and they are expecting up to two pages of matches for Dr. Kurtzberg to pick from, this also raised our spirits. Noah has not been eating very well because he has been so “snowed” from all the tests this week. He has also come down with Thrush so we have now added another medication to the long list. Noah is not the most cooperative medicine taker in the world (see photo) We will be taking him in every day this weekend to have his weight checked. This is because he has not been eating well, so they will need to check him for dehydration. We are in good hands here and are so thankful for the skill and determination of the Duke Bone Marrow Transplant Team. We appreciate all the messages and prayers that pour into his web site daily. God continues to work miracles through this little boy. We are so blessed.

Thursday, January 23, 2003 at 07:09 PM (CST)

Entry 4
It snowed last night and everything came to a stand still. All of Noah’s appointments were cancelled except for his MRI. We talked to the nurse coordinator today and it looks like Noah will not begin chemotherapy until the 3rd of February, with his stem cell transplant happening on the 12th. The main reason for the delay is room on the ward and the testing of Noah’s stem cell donor. They have to make sure that the stem cells they use do not contain the carrier version of the gene that gave him the dieses. The testing of the donor cells will take five days. We have some anxiety and defiantly no patience because we are fighting a battle against the clock, and every day lost more of Noah’s brain is damaged. We do understand though how important it is to get it right the first time.
Tomorrow Noah gets his nerve conduction study. They will be seeing if there is any delay or damage to his nerves by measuring the amount of time it takes for an electric charge to travel the length of his body. I have had this done to me, so I know it will be uncomfortable for him. We will then meet with his Nurse Practitioner who will go over the stem cell transplant with us and field any questions. This kid has been such a trooper, he has put up with a lot of poking and prodding. He didn’t even cry today when they suctioned his nose for his viral test. (We added no new photos today)

Wednesday, January 22, 2003 at 06:13 PM (CST)

Entry 3
Noah went to the Center for Development and Learning at the University of North Carolina and was tested by Dr. Escolar, who does research for the Hunter’s Hope Foundation. She checked the range of movement of his arms and legs and tested his reflexes. She told us that she didn’t think Noah would ever walk, but that he would likely be intellectually normal and may require some speech therapy later on. Noah then came back to Duke and was given an EEG and Evoked Potential Studies, which will take a day or two to interpret (see photos).
Noah’s Thursday is jam packed all day. He will be receiving a respiratory viral battery first thing tomorrow morning. We then have to take him to Audiology, and after that ophthalmology. The rest of the day will be spent in MRI where they will be looking at the damage caused by the disease and seeing where his myelin is in his brain. For the last test they are going to sedate him so he should sleep well tomorrow night.

Wednesday, January 22, 2003 at 03:50 PM (CST)

Entry 3
Noah went to the Center for Development and Learning at the University of North Carolina and was tested by Dr. Escolar who does research for the Hunters Hope Foundation. She checked the range of movement of his arms and legs and tested his reflexes. She told us that she didn’t think Noah would ever walk, but that he would likely be intellectually normal and may require some speech therapy later on. Noah then came back to Duke and was given an EEG and Evoked Potential Studies, which will take a day or two to interpret (see photos).
Noah’s Thursday is jam packed all day. He will be receiving a respiratory viral battery first thing tomorrow morning. We then have to take him to Audiology and after that ophthalmology. The reset of the day will be spent in MRI were they will be looking at the damage caused by the diseases and seeing were his Milam is in his brain. For the last test they are going to sedate him so he should sleep well tomorrow night.

Tuesday, January 21, 2003 at 07:04 PM (CST)

Entry 2
Today Noah met with Dr. Kurtzberg and her team. He was examined by his Nurse Practitioner (Bridget) and then sent to the clinic to be weighted and measured. We then took him to the Cardio Clinic were they checked his lung capacity. He did great! Everything checked out, as it should of. His blood is being matched and they should have his stem cells tested and ready by the end of the week. On Tuesday of next week Noah will get his center line and should start chemo soon after that. Noah will be going to the Center for Development and Learning at the University of North Carolina tomorrow to determine what damage the disease has done to his development. He will then come back to Duke for an Evoked Potential Study. This is to measure how his nervous system deals with sight, sound, and skin sensation stimulus and will help the doctor determine if any one area of his brain has received more damage then the other. Doctor Kurtzberg changed Noah’s medications today to Vioxx, which is an anti-inflammatory that will relive some of the inflammation of the brain and may slow the damage done by the diseases. She also subscribed Ativan, which helps to calm him when he becomes agitated, a symptom of the diseases. Noah is doing great, he was the perfect gentleman during a long and difficult day. We appreciate all the love and support from everyone. Continue to check the web page, Debbie or my self will update it as often as possible.

Sunday, January 19, 2003 at 04:58 AM (CST)

Our first day in North Carolina. Noah is doing great. Eating well and laughing and smiling. Tuesday he will see Dr. Kurtzberg and the process will begin.

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